Bleeding diathesis: Difference between revisions
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{{Bleeding diathesis}} | {{Bleeding diathesis}} | ||
{{CMG}}; {{AE}} {{MJ}}, {{N.F}} | {{CMG}}; {{AE}} {{MJ}}, {{N.F}}, {{S.G.}} | ||
{{SK}} Hypocoagulopathy; blood coagulation disorders; hemorrhagic diathesis; hemostasis abnormality; bleeding tendency | |||
== Overview == | == Overview == | ||
Bleeding diathesis is | Bleeding diathesis is susceptibility to bleed due to [[Coagulopathy|coagulopathy disorders]] or [[platelets]] disorders. These diseases can occur due to a disorder of [[homeostasis]], localized process ([[tissue]] injury), or [[Medication|medications]]. Bleeding diathesis can be resulted from [[vessel wall]] injury, [[platelet]] disorders, and [[coagulation factor]] disorders. Clinical manifestation of bleeding disorders can have a wide range of symptoms from asymptomatic to symptomatic massive and life threatening bleeding. [[Platelet]] disorders mostly have skin manifestations such as [[Petechia|petechiae]], and [[ecchymoses]]. In order to find the cause of hypo-[[coagulopathy]]; there are established laboratory tests, such as [[peripheral blood smear]], [[platelet]] count and [[platelet]] function analysis, [[coagulation factor]] deficiencies and inhibitors, [[fibrinolysis]] tests (eg. [[D-dimer]] level), [[bleeding time|bleeding time (BT)]], [[prothrombin time|prothrombin time (PT)]], [[Partial thromboplastin time|activated partial thromboplastin time (aPTT)]], [[thrombin time]] (TT), and reptilase time. In the case of any abnormal bleeding, first line of screening tests are [[Complete blood count|CBC]], [[PT]], [[Partial thromboplastin time|PTT]], [[Bleeding time|BT]], and [[Thrombin time|TT]]. | ||
== Classification == | == Classification == | ||
Disorders of hemostasis can be classified into two main categories: platelet disorders, and disorders of coagulation. Each category can be further classified as bellow: | Disorders of hemostasis can be classified into two main categories: platelet disorders, and disorders of coagulation. Each category can be further classified as bellow: | ||
{{Family tree/start}} | |||
{{familytree | | | | | | | | | | | | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | | | A01=Abnormal hemostasis }} | |||
{{familytree | | | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | }} | |||
{{familytree | | | | | | | | | | | | | | | | | | | | | | | | B01 | | | | | | | | | | | | | | | | | |B01=<br>• Patient history-sign & symptom: deep soft tissue & mucocutaneus bleeding <br>• Screen test CBC-Plt-PT&PTT-BT-TT }} | |||
{{familytree | | | | | | | | | | | | | | |,|-|-|-|-|-|-|-|-|-|^|-|-|-|-|-|-|-|-|-|-|-|-|.| | | | | | | | | }} | |||
{{familytree | | | | | | | | | | | | | | B01 | | | | | | | | | | | | | | | | | | | | | B02 | | | | | | | | |B01=Hx of deep soft tissue bleeding |B02=Hx of mucocotaneus bleeding | | | }} | |||
{{familytree | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | }} | |||
{{familytree | | | | | | | | | | | | | | C01 | | | | | | | | | | | | | | | | | | | | | | C02 | | | | | | | | |C01=Coagulopathy |C02=Plt disorder | | | }} | |||
{{familytree | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | | |,|-|-|^|-|.| | | | | | | | }} | |||
{{familytree | | | | | | | | | | | | | | D01 | | | | | | | | | | | | | | | | | | | D02 | | | D03 | | | | | | | |D01=Family history |D02=Normal plt count |D03=Low plt count | | | }} | |||
{{familytree | | | | |,|-|-|-|-|-|-|-|-|-|^|-|-|-|-|-|-|-|-|-|-|-|.| | | | | | | | |!| | | | | | | | | | | }} | |||
{{familytree | | | | | E01 | | | | | | | | | | | | | | | | | | | E02 | | | | | | | E03 | | | | | | |E01=(+)<br>• Inherited coagulpathy |E02=(-)<br>• Acquired coagulopathy |E03=Functional Plt disorder | | |}} | |||
{{familytree | |,|-|-|-|+|-|-|-|.| | | | |,|-|-|-|v|-|-|-|v|-|-|-|+|-|-|-|-|-|-|.| |!| | | }} | |||
{{familytree | F01 | | F02 | | F03 | | | F04 | | F05 | | F06 | | F07 | | | | | F08 |!| | | |F01=↑Plt <br>• Hemophillia <br>• VWD <br>• Factor VIII or IX deficiency |F02=↑PT <br>• Factor VII deficiency |F03=↑PT&↑PTT <br>• Fibrinogen deficiency <br>• Factor II deficiency <br>• Factor V deficiency <br>• Factor X deficiency |F04=↑PTT <br>• Factor inhibitor <br>• Anti phospholipid Ab syndrome |F05=↑PT <br>• Factor inhibitor <br>• Vit K deficiency <br>• Liver disease |F06=↑PT&↑PTT <br>• Factor inhibit <br>• DIC <br>• Liver failure <br>• Late stage of Vit K deficiency |F07=↑ Afibrinogenemia <br>• Heparin inhibitor <br>• Direct thrombin inhibitor |F08=Abnormal solobity <br>• Factor XIII deficiency <br>• Cross-linkin inhibitor | | |}} | |||
{{familytree | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | | | |!| | | | | | | | | | | }} | |||
{{familytree | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | |,|-|^|-|.| | | | | | | | | }} | |||
{{familytree | | | | | | | | | | | | | | | | | | | | | | G01 | | | | | | | | | G02 | | G03 |G01=Not corrected with mixing with NL plasma |G02=HX(+) |G03=HX(-) | | | }} | |||
{{familytree | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | |!| | | |!| | | | | |}} | |||
{{familytree | | | | | | | | | | | | | | | | | | | | | | H01 | | | | | | | | | H02 | | H03 | | |H01=<br>• Factor inhibitors <br>• Lupus anti coagulant <br>• DIC <br>• Heparin or direct thrombin inhibitors |H02=Congenital |H03=Acquired | | | }} | |||
{{Family tree/end}} | |||
== | == Differentiating Bleeding Disorders from Other Diseases == | ||
Different causes of bleeding disorders can be differentiated based on their clinical manifestation and laboratory findings. | |||
These features have discussed in the below table: | |||
{| | |||
{| | |||
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Category | ! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Category | ||
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + | | ! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Subcategory | ||
! colspan="2" rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + | | ! colspan="2" rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease | ||
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |History | ! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |History | ||
! colspan=" | ! colspan="6" align="center" style="background:#4479BA; color: #FFFFFF;" + |Clinical manifestation | ||
! colspan="5" align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory testing | ! colspan="5" align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory testing | ||
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments | ! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments | ||
|- | |- | ||
!align="center" style="background:#4479BA; color: #FFFFFF;" + |Petechia | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mucosal bleeding | ||
!align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Petechia | ||
!align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses | ||
!align="center" style="background:#4479BA; color: #FFFFFF;" + |Hematoma | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia | ||
!align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemarthrosis | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hematoma | ||
!align="center" style="background:#4479BA; color: #FFFFFF;" + | | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemarthrosis | ||
!align="center" style="background:#4479BA; color: #FFFFFF;" + | | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Plt | ||
!align="center" style="background:#4479BA; color: #FFFFFF;" + | | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |BT | ||
!align="center" style="background:#4479BA; color: #FFFFFF;" + | | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |PT | ||
!align="center" style="background:#4479BA; color: #FFFFFF;" + | | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |PTT | ||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |TT | |||
|- | |- | ||
! rowspan="16" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Platelet disorders | |||
! rowspan="7" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Thrombocytopenia]] | |||
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Infection]]-Induced [[thrombocytopenia]]<ref name="HerbingerSchunk2012">{{cite journal|last1=Herbinger|first1=K.-H.|last2=Schunk|first2=M.|last3=Nothdurft|first3=H. D.|last4=von Sonnenburg|first4=F.|last5=Löscher|first5=T.|last6=Bretzel|first6=G.|title=Comparative study on infection-induced thrombocytopenia among returned travellers|journal=Infection|volume=40|issue=4|year=2012|pages=373–379|issn=0300-8126|doi=10.1007/s15010-012-0242-9}}</ref> | |||
| | |||
| align="left" style="background:#F5F5F5;" | | |||
* History of prior infection | * History of prior infection | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
|↓ | | align="center" style="background:#F5F5F5;" | + | ||
|↑ | | align="center" style="background:#F5F5F5;" | ↓ | ||
| | | align="center" style="background:#F5F5F5;" | ↑ | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| align="center" style="background:#F5F5F5;" | − | |||
|- | |- | ||
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Medication]]-Induced [[Thrombocytopenia|Thrombocytopenia]]<ref name="EltingCantor2003">{{cite journal|last1=Elting|first1=Linda S.|last2=Cantor|first2=Scott B.|last3=Martin|first3=Charles G.|last4=Hamblin|first4=Lois|last5=Kurtin|first5=Danna|last6=Rivera|first6=Edgardo|last7=Vadhan-Raj|first7=Saroj|last8=Benjamin|first8=Robert S.|title=Cost of chemotherapy-induced thrombocytopenia among patients with lymphoma or solid tumors|journal=Cancer|volume=97|issue=6|year=2003|pages=1541–1550|issn=0008-543X|doi=10.1002/cncr.11195}}</ref> | |||
| | | align="left" style="background:#F5F5F5;" | | ||
*History of [[Medication|medications]] such as: | *History of [[Medication|medications]] such as: | ||
** [[Furosemide]] | ** [[Furosemide]] | ||
Line 87: | Line 88: | ||
** [[Sulfonamide (medicine)|Sulfonamides]] | ** [[Sulfonamide (medicine)|Sulfonamides]] | ||
** [[Linezolid]] | ** [[Linezolid]] | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
|↓ | | align="center" style="background:#F5F5F5;" | + | ||
|↑ | | align="center" style="background:#F5F5F5;" | ↓ | ||
| | | align="center" style="background:#F5F5F5;" | ↑ | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
|Most important | | align="center" style="background:#F5F5F5;" | Nl | ||
| align="center" style="background:#F5F5F5;" | Most important part of treatment is discontinuing of the medication. | |||
|- | |- | ||
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Heparin-Induced Thrombocytopenia|Heparin-Induced thrombocytopenia]]<ref name="Miller2003">{{cite journal|last1=Miller|first1=Penny L.|title=Heparin-induced Thrombocytopenia Recognition and Treatment|journal=AORN Journal|volume=78|issue=1|year=2003|pages=79–89|issn=00012092|doi=10.1016/S0001-2092(06)61348-3}}</ref> | |||
| | | align="left" style="background:#F5F5F5;" | | ||
* [[Thrombosis]] | * [[Thrombosis]] | ||
* Unexplained [[thrombocytopenia]] up to 3 weeks after the end of [[heparin]] therapy | * Unexplained [[thrombocytopenia]] up to 3 weeks after the end of [[heparin]] therapy | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| + | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
|↓ | | align="center" style="background:#F5F5F5;" | + | ||
|↑ | | align="center" style="background:#F5F5F5;" | ↓ | ||
| | | align="center" style="background:#F5F5F5;" | ↑ | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" | Nl | ||
|For more information click here: [[Heparin-induced thrombocytopenia]]. | | align="center" style="background:#F5F5F5;" | ↑ | ||
| align="center" style="background:#F5F5F5;" | For more information click here: [[Heparin-induced thrombocytopenia]]. | |||
|- | |- | ||
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Idiopathic thrombocytopenic purpura|Immune Thrombocytopenic Purpura]]<ref name="CurtisKaliszewski2006">{{cite journal|last1=Curtis|first1=Brian R.|last2=Kaliszewski|first2=James|last3=Marques|first3=Marisa B.|last4=Saif|first4=M. Wasif|last5=Nabelle|first5=Lisle|last6=Blank|first6=Jules|last7=McFarland|first7=Janice G.|last8=Aster|first8=Richard H.|title=Immune-mediated thrombocytopenia resulting from sensitivity to oxaliplatin|journal=American Journal of Hematology|volume=81|issue=3|year=2006|pages=199–201|issn=0361-8609|doi=10.1002/ajh.20516}}</ref> | |||
| | | align="left" style="background:#F5F5F5;" | | ||
* History of prior [[infection]] or no history | * History of prior [[infection]] or no history | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
|↓ | | align="center" style="background:#F5F5F5;" | + | ||
|↑ | | align="center" style="background:#F5F5F5;" | ↓ | ||
| | | align="center" style="background:#F5F5F5;" | ↑ | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| align="center" style="background:#F5F5F5;" | − | |||
|- | |- | ||
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Inherited [[Thrombocytopenia]]<ref name="Drachman2004">{{cite journal|last1=Drachman|first1=J. G.|title=Inherited thrombocytopenia: when a low platelet count does not mean ITP|journal=Blood|volume=103|issue=2|year=2004|pages=390–398|issn=0006-4971|doi=10.1182/blood-2003-05-1742}}</ref> | |||
| | | align="left" style="background:#F5F5F5;" | | ||
* Positive family history | |||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
|↓ | | align="center" style="background:#F5F5F5;" | + | ||
|↑ | | align="center" style="background:#F5F5F5;" | ↓ | ||
| | | align="center" style="background:#F5F5F5;" | ↑ | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| align="center" style="background:#F5F5F5;" | − | |||
|- | |- | ||
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Thrombotic thrombocytopenic purpura|Thrombotic Thrombocytopenic Purpura]]<ref name="George2006">{{cite journal|last1=George|first1=James N.|title=Thrombotic Thrombocytopenic Purpura|journal=New England Journal of Medicine|volume=354|issue=18|year=2006|pages=1927–1935|issn=0028-4793|doi=10.1056/NEJMcp053024}}</ref> | |||
|History of: | | align="left" style="background:#F5F5F5;" | History of: | ||
*[[Cancer]] | *[[Cancer]] | ||
*[[Bone marrow transplantation]] | *[[Bone marrow transplantation]] | ||
Line 154: | Line 159: | ||
**Immunosuppressants ([[cyclosporine]], [[mitomycin]], [[tacrolimus]]/FK506, [[interferon|interferon-α]]) | **Immunosuppressants ([[cyclosporine]], [[mitomycin]], [[tacrolimus]]/FK506, [[interferon|interferon-α]]) | ||
*[[HIV-1]] infection | *[[HIV-1]] infection | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
|↓ | | align="center" style="background:#F5F5F5;" | + | ||
|↑ | | align="center" style="background:#F5F5F5;" | ↓ | ||
| | | align="center" style="background:#F5F5F5;" | ↑ | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| align="center" style="background:#F5F5F5;" | − | |||
|- | |- | ||
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Hemolytic-uremic syndrome|Hemolytic Uremic Syndrome]]<ref name="Noris2005">{{cite journal|last1=Noris|first1=M.|title=Hemolytic Uremic Syndrome|journal=Journal of the American Society of Nephrology|volume=16|issue=4|year=2005|pages=1035–1050|issn=1046-6673|doi=10.1681/ASN.2004100861}}</ref> | |||
|History of: | | align="left" style="background:#F5F5F5;" |History of: | ||
* Infections | * [[Infections]] | ||
* [[Malignancy]], [[chemotherapy]], and [[ionizing radiation]] | |||
* [[Malignancy]], | * [[Calcineurin inhibitor]]s and [[transplantation]] | ||
* [[Calcineurin inhibitor]] | * [[Pregnancy]], [[HELLP syndrome]], and [[oral contraceptive pill]] | ||
* [[Pregnancy]], [[HELLP syndrome]] and [[oral contraceptive pill]] | * [[Systemic lupus erythematosis]] | ||
* [[Systemic lupus erythematosis]] | * [[Antiphospholipid syndrome|Antiphospholipid antibody syndrome]] | ||
* [[Glomerulopathy]] | * [[Glomerulopathy]] | ||
| align="center" style="background:#F5F5F5;" | + | |||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
|↓ | | align="center" style="background:#F5F5F5;" | ↓ | ||
|↑ | | align="center" style="background:#F5F5F5;" | ↑ | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | − | ||
|- | |- | ||
|align="center" style="padding: 5px 5px; background: #DCDCDC;" |Thromobcytosis | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Subcategory | ||
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mucosal bleeding | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Petechia | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hematoma | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemarthrosis | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Plt | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |BT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PTT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |TT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments | |||
|- | |||
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |Thromobcytosis | |||
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Iron deficiency anemia|Iron deficiency anemia]] | |||
Inflammatory diseases | Inflammatory diseases | ||
[[Splenectomy]] | [[Splenectomy]] | ||
[[Essential thrombocytosis| | |||
| | [[Essential thrombocytosis|Essential thrombocytosis]] | ||
* Digital pain | | align="left" style="background:#F5F5F5;" | | ||
* Digital [[pain]] | |||
* [[Gangrene]] | * [[Gangrene]] | ||
* [[Erythromelalgia]] | * [[Erythromelalgia]] | ||
* | * H[[eadache]] | ||
* [[Paresthesia|Paresthesias]] | * [[Paresthesia|Paresthesias]] | ||
* [[Transient ischemic attack|Transient ischemic attacks]] | * [[Transient ischemic attack|Transient ischemic attacks]] | ||
|− | | align="center" style="background:#F5F5F5;" | − | ||
|− | | align="center" style="background:#F5F5F5;" | − | ||
|− | | align="center" style="background:#F5F5F5;" | − | ||
| | | align="center" style="background:#F5F5F5;" | − | ||
| | | align="center" style="background:#F5F5F5;" |± | ||
|↑ | | align="center" style="background:#F5F5F5;" |± | ||
| | | align="center" style="background:#F5F5F5;" | ↑ | ||
| | | align="center" style="background:#F5F5F5;" | Nl or ↑ | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| align="center" style="background:#F5F5F5;" | − | |||
|- | |- | ||
! rowspan="6" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Qualitative Disorders of [[Platelet]] Function | |||
! rowspan="4" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Inherited Disorders of [[Platelet]] Function | |||
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Glanzmann's thrombasthenia|Glanzmann’s thrombasthenia]] | |||
| | | align="left" style="background:#F5F5F5;" | | ||
* Positive family history | * Positive family history | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
|Rare | | align="center" style="background:#F5F5F5;" | − | ||
| | | align="center" style="background:#F5F5F5;" | Rare | ||
|↑ | | align="center" style="background:#F5F5F5;" | Nl or ↓ | ||
| | | align="center" style="background:#F5F5F5;" | ↑ | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| align="left" style="background:#F5F5F5;" | | |||
* AR inheritance | * AR inheritance | ||
* Absence of the platelet Gp IIb/IIIa receptor | * Absence of the platelet Gp IIb/IIIa receptor | ||
* Diminished for GP 2B-3A on [[Flow cytometry| | * Diminished for GP 2B-3A on [[Flow cytometry|flow cytometry]] | ||
|- | |- | ||
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Bernard-Soulier syndrome]] | |||
| | | align="left" style="background:#F5F5F5;" | | ||
* Positive family history | * Positive family history | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | − | ||
| | | align="center" style="background:#F5F5F5;" | − | ||
|↑ | | align="center" style="background:#F5F5F5;" | Nl or ↓ | ||
| | | align="center" style="background:#F5F5F5;" | ↑ | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| align="left" style="background:#F5F5F5;" | | |||
* AR inheritance | * AR inheritance | ||
* Absence of the platelet Gp Ib-IX-V receptor | * Absence of the platelet Gp Ib-IX-V receptor | ||
Line 251: | Line 278: | ||
* Ristocetin - no aggregation | * Ristocetin - no aggregation | ||
|- | |- | ||
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Wiskott-Aldrich syndrome]] | |||
| align="left" style="background:#F5F5F5;" | | |||
* Positive family history | |||
| align="center" style="background:#F5F5F5;" | + | |||
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki> | |||
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki> | |||
| align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki> | |||
| align="center" style="background:#F5F5F5;" | − | |||
| align="center" style="background:#F5F5F5;" | − | |||
| align="center" style="background:#F5F5F5;" | Nl or ↓ | |||
| align="center" style="background:#F5F5F5;" | ↑ | |||
| align="center" style="background:#F5F5F5;" | Nl | |||
| align="center" style="background:#F5F5F5;" | Nl | |||
| align="center" style="background:#F5F5F5;" | Nl | |||
| align="left" style="background:#F5F5F5;" | | |||
* Anti-WASP antibody can be used to detect presence or absence of WAS protein | |||
* In Wiskott–Aldrich syndrome, the [[Platelet|platelets]] are small and do not function properly. They are removed by the [[spleen]], which leads to low [[platelet]] counts. | |||
|- | |||
! align="left" style="padding: 5px 5px; background: #DCDCDC;" |Platelet storage pool disorder: | |||
*[[Hermansky-Pudlak syndrome]] | *[[Hermansky-Pudlak syndrome]] | ||
*[[Chediak-Higashi syndrome]] | *[[Chediak-Higashi syndrome]] | ||
*[[Gray platelet syndrome]] | *[[Gray platelet syndrome]] | ||
| | | align="left" style="background:#F5F5F5;" | | ||
* Positive family history | * Positive family history | ||
*Hairy | * [[Hairy cell leukemia]] | ||
* Cardiovascular bypass | * [[Cardiovascular bypass]] | ||
|<nowiki>+</nowiki> | | align="center" style="background:#F5F5F5;" | + | ||
|<nowiki>+</nowiki> | | align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki> | ||
|<nowiki>+</nowiki> | | align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki> | ||
| | | align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki> | ||
| | | align="center" style="background:#F5F5F5;" | − | ||
| | | align="center" style="background:#F5F5F5;" | − | ||
|↑ | | align="center" style="background:#F5F5F5;" | Nl or ↓ | ||
| | | align="center" style="background:#F5F5F5;" | ↑ | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| align="left" style="background:#F5F5F5;" | | |||
* AD inheritance | * AD inheritance | ||
* | * Abnormlities of platelet granule formation | ||
|- | |- | ||
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Acquired Disorders of [[Platelet]] Function | |||
|align=" | | align="left" style="background:#F5F5F5 " | | ||
* [[Chronic renal failure pathophysiology|Uremia]] | * [[Chronic renal failure pathophysiology|Uremia]] | ||
* Cardiopulmonary bypass | * [[Cardiopulmonary bypass]] | ||
* Hematologic disorders such as: [[Myeloproliferative disease|myeloproliferative]] and [[Myelodysplastic syndrome|myelodysplastic syndromes]] | * Hematologic disorders such as: [[Myeloproliferative disease|myeloproliferative]] and [[Myelodysplastic syndrome|myelodysplastic syndromes]] | ||
|<nowiki>+</nowiki> | | align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki> | ||
|<nowiki>+</nowiki> | | align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki> | ||
|<nowiki>+</nowiki> | | align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki> | ||
| +/ | | align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki> | ||
| | | align="center" style="background:#F5F5F5;" |± | ||
| | | align="center" style="background:#F5F5F5;" |± | ||
|↑ | | align="center" style="background:#F5F5F5;" | Nl or ↓ | ||
| | | align="center" style="background:#F5F5F5;" | ↑ | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
| align="center" style="background:#F5F5F5;" | − | |||
|- | |- | ||
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Von Willebrand disease|Von Willebrand Disease]] | |||
| align="left" style="background:#F5F5F5;" | | |||
| | |||
* Easy bruising | * Easy bruising | ||
* [[Epistaxis]] | * [[Epistaxis]] | ||
Line 299: | Line 345: | ||
* [[Menorrhagia]] | * [[Menorrhagia]] | ||
* [[Postpartum hemorrhage]] | * [[Postpartum hemorrhage]] | ||
|<nowiki>+</nowiki> | | align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki> | ||
|<nowiki>+</nowiki> | | align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki> | ||
|<nowiki>+</nowiki> | | align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki> | ||
| +/ | | align="center" style="background:#F5F5F5;" | <nowiki>+</nowiki> | ||
| | | align="center" style="background:#F5F5F5;" |± | ||
|↑ | | align="center" style="background:#F5F5F5;" |± | ||
| | | align="center" style="background:#F5F5F5;" | ↑ | ||
|↑ | | align="center" style="background:#F5F5F5;" | Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" | ↑ | ||
| | | align="center" style="background:#F5F5F5;" | ↑ | ||
|See the table below for the details about | | align="center" style="background:#F5F5F5;" | Nl | ||
| align="center" style="background:#F5F5F5;" | See the table below for the details about different types. | |||
|- | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Subcategory | |||
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mucosal bleeding | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Petechia | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hematoma | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemarthrosis | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Plt | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |BT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PTT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |TT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments | |||
|- | |- | ||
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Vessel wall disorders | |||
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Metabolism|Metabolic]] and [[Inflammation|Inflammatory]] Disorders | |||
| | ! colspan="2" align="left" style="padding: 5px 5px; background: #DCDCDC;" | | ||
* Acute febrile illnesses | * Acute febrile illnesses | ||
* [[Cryoglobulinemia|Mixed cryoglobulinemia]] | * [[Cryoglobulinemia|Mixed cryoglobulinemia]] | ||
* [[Monoclonal gammopathy|Monoclonal gammopathies]] | * [[Monoclonal gammopathy|Monoclonal gammopathies]] | ||
* | * [[Rocky Mountain spotted fever]] | ||
* [[Vitamin C]] | * [[Vitamin C deficiency]] | ||
* [[Cushing's syndrome|Cushing’s syndrome]] | * [[Cushing's syndrome|Cushing’s syndrome]] | ||
* Chronic [[glucocorticoid]] therapy | * Chronic [[glucocorticoid]] therapy | ||
* [[Vasculitis]] such as Henoch-Schönlein Purpura | |||
* [[Vasculitis]] such as Henoch-Schönlein | | align="left" style="background:#F5F5F5;" | | ||
| | * History of the underlying disease | ||
* History of the underlying disease | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" |± | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" |Nl or ↑ | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
|- | |- | ||
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |Inherited Disorders of the [[Vessel wall|Vessel Wall]] | |||
| | ! colspan="2" align="left" style="background:#DCDCDC;" | | ||
* [[Marfan's syndrome|Marfan’s syndrome]] | * [[Marfan's syndrome|Marfan’s syndrome]] | ||
* [[Ehlers-Danlos syndrome]] | * [[Ehlers-Danlos syndrome]] | ||
* [[Pseudoxanthoma elasticum]] | * [[Pseudoxanthoma elasticum]] | ||
* [[Hereditary hemorrhagic telangiectasia]] ([[Hereditary hemorrhagic telangiectasia|HHT]], or [[Osler-Weber-Rendu|Osler-Weber-Rendu disease]]) | * [[Hereditary hemorrhagic telangiectasia]] ([[Hereditary hemorrhagic telangiectasia|HHT]], or [[Osler-Weber-Rendu|Osler-Weber-Rendu disease]]) | ||
| | | align="left" style="background:#F5F5F5;" | | ||
* Positive family history | * Positive family history | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" |± | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
|↑ | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |Nl or ↑ | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| align="center" style="background:#F5F5F5;" |− | |||
|- | |- | ||
! rowspan="15" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Coagulation factor disorders | |||
<ref name="pmid28966616" /> | |||
! align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Fibrinogen]] deficiency | |||
! colspan="2" align="left" style="padding: 5px 5px; background: #DCDCDC;" | | |||
Different types of the [[fibrinogen]] disorders: | |||
* [[Fibrinogen#Congenital afibrinogenemia|Congenital afibrinogenemia]] | * [[Fibrinogen#Congenital afibrinogenemia|Congenital afibrinogenemia]] | ||
* [[Fibrinogen#Congenital hypofibrinogenemia|Congenital hypofibrinogenemia]] | * [[Fibrinogen#Congenital hypofibrinogenemia|Congenital hypofibrinogenemia]] | ||
Line 366: | Line 432: | ||
* [[Fibrinogen#Hereditary fibrinogen A.CE.B1-Chain amyloidosis|Hereditary fibrinogen Aα-Chain amyloidosis]] | * [[Fibrinogen#Hereditary fibrinogen A.CE.B1-Chain amyloidosis|Hereditary fibrinogen Aα-Chain amyloidosis]] | ||
* [[Fibrinogen#Acquired dysfibrinogenemia|Acquired dysfibrinogenemia]] | * [[Fibrinogen#Acquired dysfibrinogenemia|Acquired dysfibrinogenemia]] | ||
| align="left" style="background:#F5F5F5;" | | |||
| | |||
* [[Epistaxis]] | * [[Epistaxis]] | ||
* Easy [[Bruise|bruising]] | * Easy [[Bruise|bruising]] | ||
Line 376: | Line 440: | ||
* [[Bleeding]] from the [[umbilical cord]] stump after birth | * [[Bleeding]] from the [[umbilical cord]] stump after birth | ||
* Bleeding after [[dental surgery]] or tooth extraction | * Bleeding after [[dental surgery]] or tooth extraction | ||
* | * AbNl bleeding during or after injury, surgery, or childbirth | ||
* [[Gastrointestinal tract|Gastrointestinal]] [[hemorrhage]] | * [[Gastrointestinal tract|Gastrointestinal]] [[hemorrhage]] | ||
* [[Cerebral hemorrhage]] | * [[Cerebral hemorrhage]] | ||
* [[Thrombosis]] | * [[Thrombosis]] | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| + | | align="center" style="background:#F5F5F5;" |− | ||
| + | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| + | | align="center" style="background:#F5F5F5;" |± | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
|↑ | | align="center" style="background:#F5F5F5;" |Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" |↑ | ||
|↑ | | align="center" style="background:#F5F5F5;" |↑ | ||
|↑ | | align="center" style="background:#F5F5F5;" |↑ | ||
| | | align="center" style="background:#F5F5F5;" |↑ | ||
* Impaired fibrin cross linking or clot dissolution | | align="left" style="background:#F5F5F5;" | | ||
* Impaired fibrin cross-linking or clot dissolution | |||
* | * Mild or severe bleeding depend on levels of functional fibrinogen | ||
* Variable age of onset | |||
|- | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Subcategory | |||
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mucosal bleeding | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Petechia | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hematoma | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemarthrosis | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Plt | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |BT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PTT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |TT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments | |||
|- | |- | ||
! colspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Prothrombin deficiency]] | |||
| | | align="left" style="background:#F5F5F5;" | | ||
* [[Epistaxis]] | * [[Epistaxis]] | ||
* Soft-tissue hemorrhage | * Soft-tissue hemorrhage | ||
Line 406: | Line 485: | ||
* [[Hemarthrosis]] | * [[Hemarthrosis]] | ||
* [[Intracranial hemorrhage|Intracranial]] bleeding | * [[Intracranial hemorrhage|Intracranial]] bleeding | ||
| | | align="center" style="background:#F5F5F5;" | − | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" | Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" | ↑ | ||
|↑ | | align="center" style="background:#F5F5F5;" | ↑ | ||
| | | align="center" style="background:#F5F5F5;" | ↑ | ||
| align="center" style="background:#F5F5F5;" | − | |||
|- | |- | ||
! colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor V deficiency]] | |||
| | | align="left" style="background:#F5F5F5;" | | ||
* Excessive bruising with minor injuries | * Excessive bruising with minor injuries | ||
* [[Epistaxis]] | * [[Epistaxis]] | ||
Line 426: | Line 506: | ||
* [[Intracerebral hemorrhage|Intracerebral hemorrhages]] | * [[Intracerebral hemorrhage|Intracerebral hemorrhages]] | ||
* [[Pulmonary hemorrhage]] | * [[Pulmonary hemorrhage]] | ||
| | | align="center" style="background:#F5F5F5;" | − | ||
| | | align="center" style="background:#F5F5F5;" | − | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| + | | align="center" style="background:#F5F5F5;" | + | ||
| + | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
|↑ | | align="center" style="background:#F5F5F5;" |Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" |↑ | ||
|↑ | | align="center" style="background:#F5F5F5;" |↑ | ||
| | | align="center" style="background:#F5F5F5;" |↑ | ||
|The severity of bleeding | | align="center" style="background:#F5F5F5;" |Nl | ||
| align="center" style="background:#F5F5F5;" | | |||
* The severity of bleeding related to the degree of factor V deficiency | |||
|- | |- | ||
! colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor VII deficiency]] | |||
| | | align="left" style="background:#F5F5F5;" | | ||
* Easy [[Bruise|bruising]] | * Easy [[Bruise|bruising]] | ||
* Mucosal bleeding | * Mucosal bleeding | ||
Line 446: | Line 528: | ||
* Soft tissue hematomas | * Soft tissue hematomas | ||
* [[Thrombosis]] | * [[Thrombosis]] | ||
| | | align="center" style="background:#F5F5F5;" | | ||
| | | align="center" style="background:#F5F5F5;" | | ||
| | | align="center" style="background:#F5F5F5;" | | ||
| + | | align="center" style="background:#F5F5F5;" | + | ||
| + | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" | | ||
| | | align="center" style="background:#F5F5F5;" |↑ | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
|Thrombosis | | align="center" style="background:#F5F5F5;" |Nl | ||
| align="left" style="background:#F5F5F5;" | | |||
* Thrombosis in inherited factor VII deficiency | |||
* Treatment with the administration of factor VII replacement therapy | |||
|- | |- | ||
! colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor X deficiency]] | |||
| | | align="left" style="background:#F5F5F5;" | | ||
* Prolonged bleeding following circumcision | * Prolonged bleeding following circumcision | ||
* Easy [[Bruise|bruising]] | * Easy [[Bruise|bruising]] | ||
* [[Hematuria]] | * [[Hematuria]] | ||
Line 471: | Line 555: | ||
* Intracranial bleeding | * Intracranial bleeding | ||
* Hemarthroses | * Hemarthroses | ||
| | | align="center" style="background:#F5F5F5;" | | ||
| + | | align="center" style="background:#F5F5F5;" | + | ||
| + | | align="center" style="background:#F5F5F5;" | + | ||
| + | | align="center" style="background:#F5F5F5;" | + | ||
| + | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" |Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" |↑ | ||
| | | align="center" style="background:#F5F5F5;" |↑ | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| align="center" style="background:#F5F5F5;" |− | |||
|- | |- | ||
! colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor XII|Factor XII deficiency]] | |||
| | | align="left" style="background:#F5F5F5;" | | ||
* | * Asymptomatic | ||
* Recurrent miscarriages | * Recurrent miscarriages | ||
* Painful leg ulcers | * Painful leg ulcers | ||
| | | align="center" style="background:#F5F5F5;" | | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |↑ | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| align="center" style="background:#F5F5F5;" | | |||
|- | |- | ||
| colspan=" | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Subcategory | ||
| | ! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease | ||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History | |||
| | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mucosal bleeding | ||
| | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Petechia | ||
| | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses | ||
| | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia | ||
| | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hematoma | ||
| | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemarthrosis | ||
| | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Plt | ||
| | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |BT | ||
| | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |PT | ||
| | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |PTT | ||
| | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |TT | ||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments | |||
|- | |- | ||
! colspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[High-molecular-weight kininogen|High molecular weight kininogen (HMWK)]] deficiency | |||
| | | align="left" style="background:#F5F5F5;" | | ||
* | * Positive family history of bleeding | ||
| | | align="center" style="background:#F5F5F5;" | | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |↑ | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| align="center" style="background:#F5F5F5;" | | |||
|- | |- | ||
| | ! colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Prekallikrein]] deficiency | ||
| align="left" style="background:#F5F5F5;" | | |||
* Positive family history of bleeding | |||
| align="center" style="background:#F5F5F5;" | | |||
| align="center" style="background:#F5F5F5;" |− | |||
| align="center" style="background:#F5F5F5;" |− | |||
| align="center" style="background:#F5F5F5;" |− | |||
| align="center" style="background:#F5F5F5;" |− | |||
| align="center" style="background:#F5F5F5;" |− | |||
| align="center" style="background:#F5F5F5;" |Nl | |||
| align="center" style="background:#F5F5F5;" |Nl | |||
| align="center" style="background:#F5F5F5;" |Nl | |||
| align="center" style="background:#F5F5F5;" |↑ | |||
| align="center" style="background:#F5F5F5;" |Nl | |||
| align="center" style="background:#F5F5F5;" | | |||
|- | |||
! rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor XIII deficiency]]<ref>{{cite journal | vauthors = Dorgalaleh A, Naderi M, Hosseini MS, Alizadeh S, Hosseini S, Tabibian S |display-authors=etal | title = Factor XIII Deficiency in Iran: A Comprehensive Review of the Literature. Seminars in thrombosis and hemostasis; | volume = 41 | issue = 3 (41) | pages = 323–329 | date=2015}}</ref> | |||
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" | | |||
* Sub unit A mutation disease (more common) | * Sub unit A mutation disease (more common) | ||
* Sub unit B mutation disease | * Sub unit B mutation disease | ||
| | | align="left" style="background:#F5F5F5;" | | ||
* | * Positive family history of bleeding | ||
| | | align="center" style="background:#F5F5F5;" |± | ||
| | | align="center" style="background:#F5F5F5;" |± | ||
| | | align="center" style="background:#F5F5F5;" |± | ||
| | | align="center" style="background:#F5F5F5;" |± | ||
| | | align="center" style="background:#F5F5F5;" |± | ||
| | | align="center" style="background:#F5F5F5;" |± | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |Nl or ↑ | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
* Impaired fibrin cross linking or clot dissolution | | align="left" style="background:#F5F5F5;" | | ||
* Impaired fibrin cross-linking or clot dissolution | |||
* The severity of factor XIII deficiency bleeds can be different in different patients | * The severity of factor XIII deficiency bleeds can be different in different patients | ||
|- | |- | ||
! rowspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Hemophilia]]<ref name="pmid26897598">{{cite journal |vauthors=Peyvandi F, Garagiola I, Young G |title=The past and future of haemophilia: diagnosis, treatments, and its complications |journal=Lancet |volume=388 |issue=10040 |pages=187–97 |date=July 2016 |pmid=26897598 |doi=10.1016/S0140-6736(15)01123-X |url=}}</ref> | |||
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Type A deficiency | |||
| align="left" style="background:#F5F5F5;" | | |||
| | |||
* Eeasy [[Bruise|bruising]] | * Eeasy [[Bruise|bruising]] | ||
* Inadequate clotting in [[trauma]] or mild injury | * Inadequate clotting in [[trauma]] or mild injury | ||
Line 560: | Line 664: | ||
* [[Epistaxis]] | * [[Epistaxis]] | ||
* [[Gingival bleeding]] | * [[Gingival bleeding]] | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| + | | align="center" style="background:#F5F5F5;" |− | ||
| + | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |↑ | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| align="center" style="background:#F5F5F5;" |− | |||
|- | |- | ||
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Type B deficiency | |||
| | | align="left" style="background:#F5F5F5;" | | ||
* Neonatal bleeding | * Neonatal bleeding | ||
* Trauma-related soft | * Trauma-related soft tissue hemorrhage | ||
* [[Hemarthrosis]] | * [[Hemarthrosis]] | ||
* [[Hematoma|Hematomas]] | * [[Hematoma|Hematomas]] | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| + | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |↑ | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| align="center" style="background:#F5F5F5;" |− | |||
|- | |- | ||
! colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Type C deficiency | |||
| | | align="left" style="background:#F5F5F5;" | | ||
* | * Positive family history | ||
* Bleeding after surgery or injury | * Bleeding after surgery or injury | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
| | | align="center" style="background:#F5F5F5;" |− | ||
|Rare | | align="center" style="background:#F5F5F5;" | + | ||
|Rare | | align="center" style="background:#F5F5F5;" |Rare | ||
| | | align="center" style="background:#F5F5F5;" |Rare | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" |Nl | ||
| | | align="center" style="background:#F5F5F5;" |↑ | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| align="center" style="background:#F5F5F5;" |− | |||
|- | |- | ||
| | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Subcategory | ||
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease | |||
| | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |History | ||
| | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Mucosal bleeding | ||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Petechia | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Ecchymoses | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Menorrhagia | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hematoma | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemarthrosis | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Plt | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |BT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |PTT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |TT | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments | |||
|- | |||
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Rare diseases | |||
! colspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Disseminated intravascular coagulation|Disseminated Intravascular Coagulation]]<ref name="pmid580488">{{cite journal |vauthors=Siegal T, Seligsohn U, Aghai E, Modan M |title=Clinical and laboratory aspects of disseminated intravascular coagulation (DIC): a study of 118 cases |journal=Thromb. Haemost. |volume=39 |issue=1 |pages=122–34 |date=February 1978 |pmid=580488 |doi= |url=}}</ref><ref name="pmid19222477">{{cite journal |vauthors=Levi M, Toh CH, Thachil J, Watson HG |title=Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology |journal=Br. J. Haematol. |volume=145 |issue=1 |pages=24–33 |date=April 2009 |pmid=19222477 |doi=10.1111/j.1365-2141.2009.07600.x |url=}}</ref><ref name="pmid18066597">{{cite journal |vauthors=Ghosh K, Shetty S |title=Blood coagulation in falciparum malaria--a review |journal=Parasitol. Res. |volume=102 |issue=4 |pages=571–6 |date=March 2008 |pmid=18066597 |doi=10.1007/s00436-007-0832-0 |url=}}</ref><ref name="pmid5804882">{{cite journal |vauthors=Siegal T, Seligsohn U, Aghai E, Modan M |title=Clinical and laboratory aspects of disseminated intravascular coagulation (DIC): a study of 118 cases |journal=Thromb. Haemost. |volume=39 |issue=1 |pages=122–34 |date=February 1978 |pmid=580488 |doi= |url=}}</ref><ref name="pmid1531791">{{cite journal |vauthors=Fourrier F, Chopin C, Goudemand J, Hendrycx S, Caron C, Rime A, Marey A, Lestavel P |title=Septic shock, multiple organ failure, and disseminated intravascular coagulation. Compared patterns of antithrombin III, protein C, and protein S deficiencies |journal=Chest |volume=101 |issue=3 |pages=816–23 |date=March 1992 |pmid=1531791 |doi= |url=}}</ref> | |||
| align="left" style="background:#F5F5F5;" | | |||
* [[Trauma]] | * [[Trauma]] | ||
* Burn | * [[Burn]] | ||
* [[Crush injury]] | * [[Crush injury]] | ||
* [[Sepsis]] | * [[Sepsis]] | ||
Line 617: | Line 739: | ||
* Obstetric complication: abruption, amniotic fluid embolism | * Obstetric complication: abruption, amniotic fluid embolism | ||
* [[Hemolytic anemia]] | * [[Hemolytic anemia]] | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
|↓ | | align="center" style="background:#F5F5F5;" | + | ||
|↑ | | align="center" style="background:#F5F5F5;" |↓ | ||
|↑ | | align="center" style="background:#F5F5F5;" |↑ | ||
|↑ | | align="center" style="background:#F5F5F5;" |↑ | ||
| | | align="center" style="background:#F5F5F5;" |↑ | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| align="center" style="background:#F5F5F5;" |− | |||
|- | |- | ||
! colspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Vitamin K Deficiency]]<ref name="pmid165052572">{{cite journal| author=Dezee KJ, Shimeall WT, Douglas KM, Shumway NM, O'malley PG| title=Treatment of excessive anticoagulation with phytonadione (vitamin K): a meta-analysis. | journal=Arch Intern Med | year= 2006 | volume= 166 | issue= 4 | pages= 391-7 | pmid=16505257 | doi=10.1001/.391 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16505257 }}</ref> | |||
| | | align="left" style="background:#F5F5F5;" | | ||
| | |||
* Bleeding after trauma | * Bleeding after trauma | ||
* [[Epistaxis]] | * [[Epistaxis]] | ||
Line 641: | Line 763: | ||
* Oozing from venipuncture sites | * Oozing from venipuncture sites | ||
* Easy [[Bruise|bruisability]] | * Easy [[Bruise|bruisability]] | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| + | | align="center" style="background:#F5F5F5;" |− | ||
| + | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
| | | align="center" style="background:#F5F5F5;" | + | ||
|↑ | | align="center" style="background:#F5F5F5;" |Nl | ||
|↑ | | align="center" style="background:#F5F5F5;" |↑ | ||
| | | align="center" style="background:#F5F5F5;" |↑ | ||
| | | align="center" style="background:#F5F5F5;" |Nl or mildly prolonged | ||
| | | align="center" style="background:#F5F5F5;" |Nl | ||
| align="center" style="background:#F5F5F5;" |− | |||
|} | |} | ||
==Different types of Von-Willebrand diseases== | |||
{| | |||
Different types of Von-Willebrand diseases | ! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Type of VWD | ||
!Type of VWD | ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Type of factor deficiency | ||
! | ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Prevalence | ||
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Inheritance pattern | |||
!Prevalence | ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Clinical manifestations | ||
!Inheritance pattern | ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |VWF activity | ||
!Clinical manifestations | ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |RIPA | ||
!VWF activity | ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Factor VIII | ||
!RIPA | |||
!Factor VIII | |||
|- | |- | ||
|Type 1 | ! colspan="2" align="center" style="background:#DCDCDC;" |Type 1 | ||
| | | align="center" style="background:#F5F5F5;" | Quantitative/ partial | ||
|Quantitative/ partial | | align="center" style="background:#F5F5F5;" | 60-70% | ||
|60-70% | | align="center" style="background:#F5F5F5;" | AD | ||
|AD | | align="left" style="background:#F5F5F5;" | | ||
| | |||
* Bleeding severity mild to severe | * Bleeding severity mild to severe | ||
|↓ | | align="center" style="background:#F5F5F5;" | ↓ | ||
|↓ | | align="center" style="background:#F5F5F5;" | ↓ | ||
|↓ | | align="center" style="background:#F5F5F5;" | ↓ | ||
|- | |- | ||
! rowspan="4" align="center" style="background:#DCDCDC;" |Type 2 | |||
! align="center" style="background:#DCDCDC;" |2A | |||
| align="center" style="background:#F5F5F5;" | Qualitative | |||
| align="center" style="background:#F5F5F5;" | 10% | |||
|AD/AR | | align="center" style="background:#F5F5F5;" | AD/AR | ||
| | | align="left" style="background:#F5F5F5;" | | ||
* Moderate to severe bleeding | * Moderate to severe [[bleeding]] | ||
|↓ | | align="center" style="background:#F5F5F5;" | ↓ | ||
|↓ | | align="center" style="background:#F5F5F5;" | ↓ | ||
|N or ↓ | | align="center" style="background:#F5F5F5;" | N or ↓ | ||
|- | |- | ||
|2B | ! align="center" style="background:#DCDCDC;" |2B | ||
|Qualitative | | align="center" style="background:#F5F5F5;" | Qualitative | ||
|5% | | align="center" style="background:#F5F5F5;" | 5% | ||
|AD | | align="center" style="background:#F5F5F5;" | AD | ||
| | | align="left" style="background:#F5F5F5;" | | ||
* [[Thrombocytopenia]] | * [[Thrombocytopenia]] | ||
* Moderate to severe bleeding | * Moderate to severe [[bleeding]] | ||
|↓ | | align="center" style="background:#F5F5F5;" | ↓ | ||
|↑ | | align="center" style="background:#F5F5F5;" | ↑ | ||
|N | | align="center" style="background:#F5F5F5;" | N or ↓ | ||
|- | |- | ||
|2M | ! align="center" style="background:#DCDCDC;" |2M | ||
|Qualitative | | align="center" style="background:#F5F5F5;" | Qualitative | ||
|<1% | | align="center" style="background:#F5F5F5;" | <1% | ||
|AD/AR | | align="center" style="background:#F5F5F5;" | AD/AR | ||
| | | align="left" style="background:#F5F5F5;" | | ||
* Moderate to severe bleeding | * Moderate to severe [[bleeding]] | ||
|↓ | | align="center" style="background:#F5F5F5;" | ↓ | ||
|↓ | | align="center" style="background:#F5F5F5;" | ↓ | ||
|N or ↓ | | align="center" style="background:#F5F5F5;" | N or ↓ | ||
|- | |- | ||
|2N | ! align="center" style="background:#DCDCDC;" |2N | ||
|Qualitative | | align="center" style="background:#F5F5F5;" | Qualitative | ||
|<1% | | align="center" style="background:#F5F5F5;" | <1% | ||
|AR | | align="center" style="background:#F5F5F5;" | AR | ||
| | | align="left" style="background:#F5F5F5;" | | ||
* Clinically similar to [[hemophilia A]] with joint, soft tissue, urinary bleeding | * Clinically similar to [[hemophilia A]] with [[joint]], [[soft tissue]], [[urinary]] [[bleeding]] | ||
|N | | align="center" style="background:#F5F5F5;" | N | ||
|N | | align="center" style="background:#F5F5F5;" | N | ||
|↓ | | align="center" style="background:#F5F5F5;" | ↓ | ||
|- | |- | ||
|Type 3 | ! colspan="2" align="center" style="background:#DCDCDC;" |Type 3 | ||
| | | align="center" style="background:#F5F5F5;" | Complete deficiency | ||
|Complete deficiency | | align="center" style="background:#F5F5F5;" | 1-2% | ||
|1-2% | | align="center" style="background:#F5F5F5;" | AR | ||
|AR | | align="left" style="background:#F5F5F5;" | | ||
| | * Clinically similar to [[hemophilia A]] with [[joint]] and [[soft tissue]] [[bleeding]] | ||
* Clinically similar to [[hemophilia A]] with joint and soft tissue bleeding | |||
* Severe [[mucosal bleeding]] | * Severe [[mucosal bleeding]] | ||
|Absent | | align="center" style="background:#F5F5F5;" | Absent | ||
|↓ | | align="center" style="background:#F5F5F5;" | ↓ | ||
| | | align="center" style="background:#F5F5F5;" | Low, 1-10% | ||
Low, 1-10% | |||
|} | |} | ||
For more information on Von Willebrand disease [[ von willebrand disease | | For more information on Von Willebrand disease, click [[ von willebrand disease | here]]. | ||
==References== | ==References== | ||
{{ | {{Reflist|2}} | ||
[[Category:Medicine]] | |||
[[Category:Hematology]] | [[Category:Hematology]] | ||
[[Category:Oncology]] | |||
[[Category:Up-To-Date]] | |||
[[Category:Emergency medicine]] |
Latest revision as of 20:38, 29 July 2020
Bleeding diathesis main page |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2], Nazia Fuad M.D., Sogand Goudarzi, MD [3]
Synonyms and keywords: Hypocoagulopathy; blood coagulation disorders; hemorrhagic diathesis; hemostasis abnormality; bleeding tendency
Overview
Bleeding diathesis is susceptibility to bleed due to coagulopathy disorders or platelets disorders. These diseases can occur due to a disorder of homeostasis, localized process (tissue injury), or medications. Bleeding diathesis can be resulted from vessel wall injury, platelet disorders, and coagulation factor disorders. Clinical manifestation of bleeding disorders can have a wide range of symptoms from asymptomatic to symptomatic massive and life threatening bleeding. Platelet disorders mostly have skin manifestations such as petechiae, and ecchymoses. In order to find the cause of hypo-coagulopathy; there are established laboratory tests, such as peripheral blood smear, platelet count and platelet function analysis, coagulation factor deficiencies and inhibitors, fibrinolysis tests (eg. D-dimer level), bleeding time (BT), prothrombin time (PT), activated partial thromboplastin time (aPTT), thrombin time (TT), and reptilase time. In the case of any abnormal bleeding, first line of screening tests are CBC, PT, PTT, BT, and TT.
Classification
Disorders of hemostasis can be classified into two main categories: platelet disorders, and disorders of coagulation. Each category can be further classified as bellow:
Abnormal hemostasis | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
• Patient history-sign & symptom: deep soft tissue & mucocutaneus bleeding • Screen test CBC-Plt-PT&PTT-BT-TT | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Hx of deep soft tissue bleeding | Hx of mucocotaneus bleeding | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Coagulopathy | Plt disorder | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Family history | Normal plt count | Low plt count | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
(+) • Inherited coagulpathy | (-) • Acquired coagulopathy | Functional Plt disorder | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
↑Plt • Hemophillia • VWD • Factor VIII or IX deficiency | ↑PT • Factor VII deficiency | ↑PT&↑PTT • Fibrinogen deficiency • Factor II deficiency • Factor V deficiency • Factor X deficiency | ↑PTT • Factor inhibitor • Anti phospholipid Ab syndrome | ↑PT • Factor inhibitor • Vit K deficiency • Liver disease | ↑PT&↑PTT • Factor inhibit • DIC • Liver failure • Late stage of Vit K deficiency | ↑ Afibrinogenemia • Heparin inhibitor • Direct thrombin inhibitor | Abnormal solobity • Factor XIII deficiency • Cross-linkin inhibitor | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Not corrected with mixing with NL plasma | HX(+) | HX(-) | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
• Factor inhibitors • Lupus anti coagulant • DIC • Heparin or direct thrombin inhibitors | Congenital | Acquired | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Differentiating Bleeding Disorders from Other Diseases
Different causes of bleeding disorders can be differentiated based on their clinical manifestation and laboratory findings.
These features have discussed in the below table:
Category | Subcategory | Disease | History | Clinical manifestation | Laboratory testing | Comments | ||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Mucosal bleeding | Petechia | Ecchymoses | Menorrhagia | Hematoma | Hemarthrosis | Plt | BT | PT | PTT | TT | ||||||
Platelet disorders | Thrombocytopenia | Infection-Induced thrombocytopenia[1] |
|
+ | + | + | + | + | + | ↓ | ↑ | Nl | Nl | Nl | − | |
Medication-Induced Thrombocytopenia[2] |
|
+ | + | + | + | + | + | ↓ | ↑ | Nl | Nl | Nl | Most important part of treatment is discontinuing of the medication. | |||
Heparin-Induced thrombocytopenia[3] |
|
+ | + | + | + | + | + | ↓ | ↑ | Nl | Nl | ↑ | For more information click here: Heparin-induced thrombocytopenia. | |||
Immune Thrombocytopenic Purpura[4] |
|
+ | + | + | + | + | + | ↓ | ↑ | Nl | Nl | Nl | − | |||
Inherited Thrombocytopenia[5] |
|
+ | + | + | + | + | + | ↓ | ↑ | Nl | Nl | Nl | − | |||
Thrombotic Thrombocytopenic Purpura[6] | History of:
|
+ | + | + | + | + | + | ↓ | ↑ | Nl | Nl | Nl | − | |||
Hemolytic Uremic Syndrome[7] | History of: | + | + | + | + | + | + | ↓ | ↑ | Nl | Nl | Nl | − | |||
Subcategory | Disease | History | Mucosal bleeding | Petechia | Ecchymoses | Menorrhagia | Hematoma | Hemarthrosis | Plt | BT | PT | PTT | TT | Comments | ||
Thromobcytosis | Iron deficiency anemia
Inflammatory diseases |
− | − | − | − | ± | ± | ↑ | Nl or ↑ | Nl | Nl | Nl | − | |||
Qualitative Disorders of Platelet Function | Inherited Disorders of Platelet Function | Glanzmann’s thrombasthenia |
|
+ | + | + | + | − | Rare | Nl or ↓ | ↑ | Nl | Nl | Nl |
| |
Bernard-Soulier syndrome |
|
+ | + | + | + | − | − | Nl or ↓ | ↑ | Nl | Nl | Nl |
| |||
Wiskott-Aldrich syndrome |
|
+ | + | + | + | − | − | Nl or ↓ | ↑ | Nl | Nl | Nl | ||||
Platelet storage pool disorder: |
|
+ | + | + | + | − | − | Nl or ↓ | ↑ | Nl | Nl | Nl |
| |||
Acquired Disorders of Platelet Function |
|
+ | + | + | + | ± | ± | Nl or ↓ | ↑ | Nl | Nl | Nl | − | |||
Von Willebrand Disease |
|
+ | + | + | + | ± | ± | ↑ | Nl | ↑ | ↑ | Nl | See the table below for the details about different types. | |||
Subcategory | Disease | History | Mucosal bleeding | Petechia | Ecchymoses | Menorrhagia | Hematoma | Hemarthrosis | Plt | BT | PT | PTT | TT | Comments | ||
Vessel wall disorders | Metabolic and Inflammatory Disorders |
|
|
− | + | + | ± | − | − | Nl | Nl or ↑ | Nl | Nl | Nl | − | |
Inherited Disorders of the Vessel Wall |
|
− | + | + | ± | − | − | Nl | Nl or ↑ | Nl | Nl | Nl | − | |||
Coagulation factor disorders | Fibrinogen deficiency |
Different types of the fibrinogen disorders: |
|
− | − | + | + | ± | + | Nl | ↑ | ↑ | ↑ | ↑ |
| |
Subcategory | Disease | History | Mucosal bleeding | Petechia | Ecchymoses | Menorrhagia | Hematoma | Hemarthrosis | Plt | BT | PT | PTT | TT | Comments | ||
Prothrombin deficiency |
|
− | + | + | + | + | + | Nl | Nl | ↑ | ↑ | ↑ | − | |||
Factor V deficiency |
|
− | − | + | + | + | + | Nl | ↑ | ↑ | ↑ | Nl |
| |||
Factor VII deficiency |
|
+ | + | + | Nl | ↑ | Nl | Nl |
| |||||||
Factor X deficiency |
|
+ | + | + | + | + | Nl | Nl | ↑ | ↑ | Nl | − | ||||
Factor XII deficiency |
|
− | − | − | − | − | Nl | Nl | Nl | ↑ | Nl | |||||
Subcategory | Disease | History | Mucosal bleeding | Petechia | Ecchymoses | Menorrhagia | Hematoma | Hemarthrosis | Plt | BT | PT | PTT | TT | Comments | ||
High molecular weight kininogen (HMWK) deficiency |
|
− | − | − | − | − | Nl | Nl | Nl | ↑ | Nl | |||||
Prekallikrein deficiency |
|
− | − | − | − | − | Nl | Nl | Nl | ↑ | Nl | |||||
Factor XIII deficiency[9] |
|
|
± | ± | ± | ± | ± | ± | Nl | Nl | Nl or ↑ | Nl | Nl |
| ||
Hemophilia[10] | Type A deficiency |
|
− | − | − | + | + | + | Nl | Nl | Nl | ↑ | Nl | − | ||
Type B deficiency |
|
− | − | − | + | + | + | Nl | Nl | Nl | ↑ | Nl | − | |||
Type C deficiency |
|
− | − | − | + | Rare | Rare | Nl | Nl | Nl | ↑ | Nl | − | |||
Subcategory | Disease | History | Mucosal bleeding | Petechia | Ecchymoses | Menorrhagia | Hematoma | Hemarthrosis | Plt | BT | PT | PTT | TT | Comments | ||
Rare diseases | Disseminated Intravascular Coagulation[11][12][13][14][15] |
|
+ | + | + | + | + | + | ↓ | ↑ | ↑ | ↑ | Nl | − | ||
Vitamin K Deficiency[16] |
|
+ | − | + | + | + | + | Nl | ↑ | ↑ | Nl or mildly prolonged | Nl | − |
Different types of Von-Willebrand diseases
Type of VWD | Type of factor deficiency | Prevalence | Inheritance pattern | Clinical manifestations | VWF activity | RIPA | Factor VIII | |
---|---|---|---|---|---|---|---|---|
Type 1 | Quantitative/ partial | 60-70% | AD |
|
↓ | ↓ | ↓ | |
Type 2 | 2A | Qualitative | 10% | AD/AR |
|
↓ | ↓ | N or ↓ |
2B | Qualitative | 5% | AD |
|
↓ | ↑ | N or ↓ | |
2M | Qualitative | <1% | AD/AR |
|
↓ | ↓ | N or ↓ | |
2N | Qualitative | <1% | AR |
|
N | N | ↓ | |
Type 3 | Complete deficiency | 1-2% | AR |
|
Absent | ↓ | Low, 1-10% |
For more information on Von Willebrand disease, click here.
References
- ↑ Herbinger, K.-H.; Schunk, M.; Nothdurft, H. D.; von Sonnenburg, F.; Löscher, T.; Bretzel, G. (2012). "Comparative study on infection-induced thrombocytopenia among returned travellers". Infection. 40 (4): 373–379. doi:10.1007/s15010-012-0242-9. ISSN 0300-8126.
- ↑ Elting, Linda S.; Cantor, Scott B.; Martin, Charles G.; Hamblin, Lois; Kurtin, Danna; Rivera, Edgardo; Vadhan-Raj, Saroj; Benjamin, Robert S. (2003). "Cost of chemotherapy-induced thrombocytopenia among patients with lymphoma or solid tumors". Cancer. 97 (6): 1541–1550. doi:10.1002/cncr.11195. ISSN 0008-543X.
- ↑ Miller, Penny L. (2003). "Heparin-induced Thrombocytopenia Recognition and Treatment". AORN Journal. 78 (1): 79–89. doi:10.1016/S0001-2092(06)61348-3. ISSN 0001-2092.
- ↑ Curtis, Brian R.; Kaliszewski, James; Marques, Marisa B.; Saif, M. Wasif; Nabelle, Lisle; Blank, Jules; McFarland, Janice G.; Aster, Richard H. (2006). "Immune-mediated thrombocytopenia resulting from sensitivity to oxaliplatin". American Journal of Hematology. 81 (3): 199–201. doi:10.1002/ajh.20516. ISSN 0361-8609.
- ↑ Drachman, J. G. (2004). "Inherited thrombocytopenia: when a low platelet count does not mean ITP". Blood. 103 (2): 390–398. doi:10.1182/blood-2003-05-1742. ISSN 0006-4971.
- ↑ George, James N. (2006). "Thrombotic Thrombocytopenic Purpura". New England Journal of Medicine. 354 (18): 1927–1935. doi:10.1056/NEJMcp053024. ISSN 0028-4793.
- ↑ Noris, M. (2005). "Hemolytic Uremic Syndrome". Journal of the American Society of Nephrology. 16 (4): 1035–1050. doi:10.1681/ASN.2004100861. ISSN 1046-6673.
- ↑
- ↑ Dorgalaleh A, Naderi M, Hosseini MS, Alizadeh S, Hosseini S, Tabibian S, et al. (2015). "Factor XIII Deficiency in Iran: A Comprehensive Review of the Literature. Seminars in thrombosis and hemostasis;". 41 (3 (41)): 323–329.
- ↑ Peyvandi F, Garagiola I, Young G (July 2016). "The past and future of haemophilia: diagnosis, treatments, and its complications". Lancet. 388 (10040): 187–97. doi:10.1016/S0140-6736(15)01123-X. PMID 26897598.
- ↑ Siegal T, Seligsohn U, Aghai E, Modan M (February 1978). "Clinical and laboratory aspects of disseminated intravascular coagulation (DIC): a study of 118 cases". Thromb. Haemost. 39 (1): 122–34. PMID 580488.
- ↑ Levi M, Toh CH, Thachil J, Watson HG (April 2009). "Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology". Br. J. Haematol. 145 (1): 24–33. doi:10.1111/j.1365-2141.2009.07600.x. PMID 19222477.
- ↑ Ghosh K, Shetty S (March 2008). "Blood coagulation in falciparum malaria--a review". Parasitol. Res. 102 (4): 571–6. doi:10.1007/s00436-007-0832-0. PMID 18066597.
- ↑ Siegal T, Seligsohn U, Aghai E, Modan M (February 1978). "Clinical and laboratory aspects of disseminated intravascular coagulation (DIC): a study of 118 cases". Thromb. Haemost. 39 (1): 122–34. PMID 580488.
- ↑ Fourrier F, Chopin C, Goudemand J, Hendrycx S, Caron C, Rime A, Marey A, Lestavel P (March 1992). "Septic shock, multiple organ failure, and disseminated intravascular coagulation. Compared patterns of antithrombin III, protein C, and protein S deficiencies". Chest. 101 (3): 816–23. PMID 1531791.
- ↑ Dezee KJ, Shimeall WT, Douglas KM, Shumway NM, O'malley PG (2006). "Treatment of excessive anticoagulation with phytonadione (vitamin K): a meta-analysis". Arch Intern Med. 166 (4): 391–7. doi:10.1001/.391. PMID 16505257.