Autoimmune hemolytic anemia differential diagnosis: Difference between revisions

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==Differentiating Autoimmune hemolytic anemia from other Diseases==
==Differentiating Autoimmune hemolytic anemia from other Diseases==
=== Differential diagnosis of "Bleeding disorders": ===
<span name="harr_c115s002s003p001"></span><span name="9100800"></span><span name="harr_c115s002s003p001"></span><span name="9100800"></span>
{| class="wikitable" align="center" style="border: 0px; font-size: 90%; margin: 3px;"
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Category
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Sub-category
! colspan="2" rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Diseases
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! colspan="5" align="center" style="background:#4479BA; color: #FFFFFF;" + |Clinical manifestation
! colspan="5" align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory testing
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Comments
|-
! align="center" style="background:#4479BA; color: #FFFFFF;" + |<nowiki>Petechia|Petechiae</nowiki>
! 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;" + |Platelet count
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Bleeding time (BT)
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Prothrombin time (PT)
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Activated partial thromboplastin time (aPTT)
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Thrombin time (TT)
|-
| rowspan="13" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[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<span name="harr_c115s002s001s002p001"></span><span name="9100754"></span><ref name="pmid21325604">{{cite journal |vauthors=Neunert C, Lim W, Crowther M, Cohen A, Solberg L, Crowther MA |title=The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia |journal=Blood |volume=117 |issue=16 |pages=4190–207 |date=April 2011 |pmid=21325604 |doi=10.1182/blood-2010-08-302984 |url=}}</ref><ref name="pmid26906627">{{cite journal |vauthors=Karimi O, Goorhuis A, Schinkel J, Codrington J, Vreden SGS, Vermaat JS, Stijnis C, Grobusch MP |title=Thrombocytopenia and subcutaneous bleedings in a patient with Zika virus infection |journal=Lancet |volume=387 |issue=10022 |pages=939–940 |date=March 2016 |pmid=26906627 |doi=10.1016/S0140-6736(16)00502-X |url=}}</ref><ref name="pmid25600600">{{cite journal |vauthors=Zammarchi L, Stella G, Mantella A, Bartolozzi D, Tappe D, Günther S, Oestereich L, Cadar D, Muñoz-Fontela C, Bartoloni A, Schmidt-Chanasit J |title=Zika virus infections imported to Italy: clinical, immunological and virological findings, and public health implications |journal=J. Clin. Virol. |volume=63 |issue= |pages=32–5 |date=February 2015 |pmid=25600600 |doi=10.1016/j.jcv.2014.12.005 |url=}}</ref>
|
* History of prior infection
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|↓
|↑
|N
|N
|N
|<nowiki>-</nowiki>
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Medication|Medications]]-Induced thrombocy<span name="harr_c115s002s001s003p001"></span><span name="9100757"></span>topenia <ref name="pmid25134884">{{cite journal |vauthors=Kam T, Alexander M |title=Drug-induced immune thrombocytopenia |journal=J Pharm Pract |volume=27 |issue=5 |pages=430–9 |date=October 2014 |pmid=25134884 |doi=10.1177/0897190014546099 |url=}}</ref><ref name="pmid23461497">{{cite journal |vauthors=Seco-Melantuche R, Delgado-Sánchez O, Álvarez-Arroyo L |title=[Incidence of drug-induced thrombocytopenia in hospitalized patients] |language=Spanish; Castilian |journal=Farm Hosp |volume=37 |issue=1 |pages=27–34 |date=2013 |pmid=23461497 |doi=10.7399/FH.2013.37.1.42 |url=}}</ref>
|
*History of [[Medication|medications]] such as:
** [[Furosemide]]
** [[Non-steroidal anti-inflammatory drug|Nonsteroidal anti-inflammatory drugs]] ([[Non-steroidal anti-inflammatory drug|NSAIDs]])
** [[Penicillin]]
** [[Quinidine]]
** [[Quinine]]
** [[Ranitidine]]
** [[Sulfonamide (medicine)|Sulfonamides]]
** [[Linezolid]]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|↓
|↑
|N
|N
|N
|Most important par of treatment is discontinuing of the medication.
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Heparin-Induced Thrombocytopenia|Heparin-Induced thrombocytopenia]]<span name="harr_c115s002s001s004p001"></span><span name="9100761"></span><ref name="pmid25629757">{{cite journal |vauthors=Warkentin TE, Safyan EL, Linkins LA |title=Heparin-induced thrombocytopenia presenting as bilateral adrenal hemorrhages |journal=N. Engl. J. Med. |volume=372 |issue=5 |pages=492–4 |date=January 2015 |pmid=25629757 |doi=10.1056/NEJMc1414161 |url=}}</ref>
|
* [[Thrombosis]]
* Unexplained [[thrombocytopenia]] up to 3 weeks after the end of [[heparin]] therapy
|<nowiki>+</nowiki>
| +
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|↓
|↑
|N
|N
|↑
|For more information click here: [[Heparin-induced thrombocytopenia]].
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Idiopathic thrombocytopenic purpura|Immune Thrombocytopenic Purpura]] ([[Idiopathic thrombocytopenic purpura|ITP]])<span name="harr_c115s002s001s005p001"></span><span name="9100771"></span><ref name="pmid8857953">{{cite journal |vauthors=Wright JF, Blanchette VS, Wang H, Arya N, Petric M, Semple JW, Chia WK, Freedman J |title=Characterization of platelet-reactive antibodies in children with varicella-associated acute immune thrombocytopenic purpura (ITP) |journal=Br. J. Haematol. |volume=95 |issue=1 |pages=145–52 |date=October 1996 |pmid=8857953 |doi= |url=}}</ref>
|
* History of prior [[infection]] or no history
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|↓
|↑
|N
|N
|N
| -
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Inherited [[Thrombocytopenia]]<span name="harr_c115s002s001s006p001"></span><span name="9100783"></span><ref name="pmid27025194">{{cite journal |vauthors=Johnson B, Fletcher SJ, Morgan NV |title=Inherited thrombocytopenia: novel insights into megakaryocyte maturation, proplatelet formation and platelet lifespan |journal=Platelets |volume=27 |issue=6 |pages=519–25 |date=September 2016 |pmid=27025194 |pmc=5000870 |doi=10.3109/09537104.2016.1148806 |url=}}</ref><ref name="pmid30103613">{{cite journal |vauthors=Wang Q, Cao L, Sheng G, Shen H, Ling J, Xie J, Ma Z, Yin J, Wang Z, Yu Z, Chen S, Zhao Y, Ruan C, Xia L, Jiang M |title=Application of High-Throughput Sequencing in the Diagnosis of Inherited Thrombocytopenia |journal=Clin. Appl. Thromb. Hemost. |volume= |issue= |pages=1076029618790696 |date=August 2018 |pmid=30103613 |doi=10.1177/1076029618790696 |url=}}</ref>
|
* Family history
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|↓
|↑
|N
|N
|N
| -
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Thrombotic thrombocytopenic purpura|Thrombotic Thrombocytopenic Purpura]] ([[Thrombotic thrombocytopenic purpura|TTP]])<span name="harr_c115s002s002s001p001"></span><span name="9100787"></span><ref name="pmid30220931">{{cite journal |vauthors=Knöbl P |title=Thrombotic thrombocytopenic purpura |journal=Memo |volume=11 |issue=3 |pages=220–226 |date=2018 |pmid=30220931 |doi=10.1007/s12254-018-0429-6 |url=}}</ref><ref name="pmid26386489">{{cite journal |vauthors=Mannucci PM, Cugno M |title=The complex differential diagnosis between thrombotic thrombocytopenic purpura and the atypical hemolytic uremic syndrome: Laboratory weapons and their impact on treatment choice and monitoring |journal=Thromb. Res. |volume=136 |issue=5 |pages=851–4 |date=November 2015 |pmid=26386489 |doi=10.1016/j.thromres.2015.09.007 |url=}}</ref>
|History of:
*[[Cancer]]
*[[Bone marrow transplantation]]
*[[Pregnancy]]
*[[Medication]]
**[[Platelet]] aggregation inhibitors ([[ticlopidine]] and [[clopidogrel]])
**Immunosuppressants ([[cyclosporine]], [[mitomycin]], [[tacrolimus]]/FK506, [[interferon|interferon-α]])
*[[HIV-1]] infection
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|↓
|↑
|N
|N
|N
| -
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Hemolytic-uremic syndrome|Hemolytic Uremic Syndrome]]<span name="harr_c115s002s002s002p001"></span><span name="9100796"></span><ref name="pmid24365375">{{cite journal |vauthors=Webster K, Schnitzler E |title=Hemolytic uremic syndrome |journal=Handb Clin Neurol |volume=120 |issue= |pages=1113–23 |date=2014 |pmid=24365375 |doi=10.1016/B978-0-7020-4087-0.00075-9 |url=}}</ref><ref name="pmid25845294">{{cite journal |vauthors=Picard C, Burtey S, Bornet C, Curti C, Montana M, Vanelle P |title=Pathophysiology and treatment of typical and atypical hemolytic uremic syndrome |journal=Pathol. Biol. |volume=63 |issue=3 |pages=136–43 |date=June 2015 |pmid=25845294 |doi=10.1016/j.patbio.2015.03.001 |url=}}</ref>
|History of:
* Infections
* [[Malignancy]], [[Cancer (disease)|cancer]] [[chemotherapy]] and [[ionizing radiation]]
* [[Calcineurin inhibitor]]<nowiki/>s and [[transplantation]]
* [[Pregnancy]], [[HELLP syndrome]] and [[oral contraceptive pill]]
* [[Systemic lupus erythematosis]] and [[Antiphospholipid syndrome|antiphospholipid antibody syndrome]]
* [[Glomerulopathy]]
* [[Familial]], not included in part 1
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|↓
|↑
|N
|N
|N
| -
|-
| 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
[[Splenectomy]]
[[Essential thrombocytosis|Essential thrombocytosis]]
|
* Digital pain
* [[Gangrene]]
* [[Erythromelalgia]]
* Headache
* [[Paresthesia|Paresthesias]] 
* [[Transient ischemic attack|Transient ischemic attacks]]
|−
|−
|−
| +/-
| +/-
|↑
|Ν/↑
|ɴ
|ɴ
|N
| -
|-
| rowspan="5" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Qualitative Disorders of [[Platelet]] Function<span name="harr_c115s002s004s001p001"></span><span name="9100803"></span>
| rowspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Inherited Disorders of [[Platelet]] Function<span name="harr_c115s002s004s001p001"></span><span name="9100803"></span>
| align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Glanzmann's thrombasthenia|Glanzmann’s thrombasthenia]]
|
* Positive family history
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| -
|Rare
|N/↓
|↑
|ɴ
|ɴ
|N
|
* AR inheritance
* Absence of the platelet Gp IIb/IIIa receptor/
* Diminished for GP 2B-3A on [[Flow cytometry|f<abbr>low cytometry</abbr>]]
|-
| align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Bernard-Soulier syndrome]]<ref name="pmid30077511">{{cite journal |vauthors=Dupuis A, Gachet C |title=Inherited platelet disorders : Management of the bleeding risk |journal=Transfus Clin Biol |volume=25 |issue=3 |pages=228–235 |date=September 2018 |pmid=30077511 |doi=10.1016/j.tracli.2018.07.003 |url=}}</ref><ref name="pmid29227167">{{cite journal |vauthors=Andres O, Henning K, Strauß G, Pflug A, Manukjan G, Schulze H |title=Diagnosis of platelet function disorders: A standardized, rational, and modular flow cytometric approach |journal=Platelets |volume=29 |issue=4 |pages=347–356 |date=June 2018 |pmid=29227167 |doi=10.1080/09537104.2017.1386297 |url=}}</ref>
|
* Positive family history
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| -
| -
|N/↓
|↑
|N
|N
|N
|
* AR inheritance
* Absence of the platelet Gp Ib-IX-V receptor
* On PBS: giant platelets
* Ristocetin - no aggregation
|-
| style="padding: 5px 5px; background: #DCDCDC;" |[[Platelet storage pool deficiency|Platelet storage pool disorder (SPD)]]:
*[[Hermansky-Pudlak syndrome]]
*[[Chediak-Higashi syndrome]]
*[[Gray platelet syndrome]]
|
* Positive family history
*Hairy-cell leukemia
* Cardiovascular bypass
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| -
| -
|N/↓
|↑
|N
|N
|N
|
* AD inheritance
* Abnormalities of platelet granule formation
|-
| align="center" style="padding: 5px 5px; background: #DCDCDC;" |Acquired Disorders of [[Platelet]] Function<span name="harr_c115s002s004s002p001"></span><span name="9100808"></span>
| style="padding: 5px 5px; background: #DCDCDC;" |
* [[Chronic renal failure pathophysiology|Uremia]]
* Cardiopulmonary bypass
* Hematologic disorders such as: [[Myeloproliferative disease|myeloproliferative]] and [[Myelodysplastic syndrome|myelodysplastic syndromes]]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| +/-
| +/-
|N/↓
|↑
|N
|N
|N
|<nowiki>-</nowiki>
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Von Willebrand disease|Von Willebrand Disease]]<span name="harr_c115s002s005p001"></span><span name="9100810"></span> <ref name="pmid25196510">{{cite journal |vauthors=Elbatarny M, Mollah S, Grabell J, Bae S, Deforest M, Tuttle A, Hopman W, Clark DS, Mauer AC, Bowman M, Riddel J, Christopherson PA, Montgomery RR, Rand ML, Coller B, James PD |title=Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project |journal=Haemophilia |volume=20 |issue=6 |pages=831–5 |date=November 2014 |pmid=25196510 |pmc=4251588 |doi=10.1111/hae.12503 |url=}}</ref><ref name="pmid25196510">{{cite journal |vauthors=Elbatarny M, Mollah S, Grabell J, Bae S, Deforest M, Tuttle A, Hopman W, Clark DS, Mauer AC, Bowman M, Riddel J, Christopherson PA, Montgomery RR, Rand ML, Coller B, James PD |title=Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project |journal=Haemophilia |volume=20 |issue=6 |pages=831–5 |date=November 2014 |pmid=25196510 |pmc=4251588 |doi=10.1111/hae.12503 |url=}}</ref><ref name="pmid16985174">{{cite journal |vauthors=Goodeve A, Eikenboom J, Castaman G, Rodeghiero F, Federici AB, Batlle J, Meyer D, Mazurier C, Goudemand J, Schneppenheim R, Budde U, Ingerslev J, Habart D, Vorlova Z, Holmberg L, Lethagen S, Pasi J, Hill F, Hashemi Soteh M, Baronciani L, Hallden C, Guilliatt A, Lester W, Peake I |title=Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD) |journal=Blood |volume=109 |issue=1 |pages=112–21 |date=January 2007 |pmid=16985174 |doi=10.1182/blood-2006-05-020784 |url=}}</ref><ref name="pmid9579642">{{cite journal |vauthors=Mammen EF, Comp PC, Gosselin R, Greenberg C, Hoots WK, Kessler CM, Larkin EC, Liles D, Nugent DJ |title=PFA-100 system: a new method for assessment of platelet dysfunction |journal=Semin. Thromb. Hemost. |volume=24 |issue=2 |pages=195–202 |date=1998 |pmid=9579642 |doi=10.1055/s-2007-995840 |url=}}</ref><ref name="pmid258585642">{{cite journal |vauthors=Bodó I, Eikenboom J, Montgomery R, Patzke J, Schneppenheim R, Di Paola J |title=Platelet-dependent von Willebrand factor activity. Nomenclature and methodology: communication from the SSC of the ISTH |journal=J. Thromb. Haemost. |volume=13 |issue=7 |pages=1345–50 |date=July 2015 |pmid=25858564 |pmc=5576173 |doi=10.1111/jth.12964 |url=}}</ref>
|
* Easy bruising
* [[Epistaxis]]
* Oral cavity bleeding
* Bleeding after dental extraction/surgery
* [[Menorrhagia]]
* [[Postpartum hemorrhage]]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| +/-
| +/-
|↑
|Ν
|↑
|↑
|N
|See the table below for the details about  types.
|-
| rowspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Vessel wall disorders
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Metabolism|Metabolic]] and [[Inflammation|Inflammatory]] Disorders
| align="left" style="padding: 5px 5px; background: #DCDCDC;" |
* Acute febrile illnesses
* [[Cryoglobulinemia|Mixed cryoglobulinemia]]
* [[Monoclonal gammopathy|Monoclonal gammopathies]]
* Certain pathogens, such as the rickettsiae causing [[Rocky Mountain spotted fever]]
* [[Vitamin C]] deficiency
* [[Cushing's syndrome|Cushing’s syndrome]]
* Chronic [[glucocorticoid]] therapy
* [[Ageing|Aging]] 
* [[Vasculitis]] such as Henoch-Schönlein,
|
* History of the underlying disease.
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
| -
| -
|N
|↑/N
|N
|N
|N
|<nowiki>-</nowiki>
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Inherited Disorders of the [[Vessel wall|Vessel Wall]]
| align="left" style="padding: 5px 5px; background: #DCDCDC;" |
* [[Marfan's syndrome|Marfan’s syndrome]]
* [[Ehlers-Danlos syndrome]]
* [[Pseudoxanthoma elasticum]]
* [[Hereditary hemorrhagic telangiectasia]] ([[Hereditary hemorrhagic telangiectasia|HHT]], or [[Osler-Weber-Rendu|Osler-Weber-Rendu disease]])
|
* Positive family history
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
| -
| -
|N
|↑/N
|N
|N
|N
|<nowiki>-</nowiki>
|-
| rowspan="12" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Coagulation factor disorders
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Fibrinogen]] deficiency
| style="padding: 5px 5px; background: #DCDCDC;" |Different types of the [[fibrinogen]] disorders:
* [[Fibrinogen#Congenital afibrinogenemia|Congenital afibrinogenemia]]
* [[Fibrinogen#Congenital hypofibrinogenemia|Congenital hypofibrinogenemia]]
* [[Fibrinogen#Fibrinogen storage disease|Fibrinogen storage disease]]
* [[Fibrinogen#Congenital dysfibrinogenemia|Congenital dysfibrinogenemia]]
* [[Fibrinogen#Hereditary fibrinogen A.CE.B1-Chain amyloidosis|Hereditary fibrinogen Aα-Chain amyloidosis]]
* [[Fibrinogen#Acquired dysfibrinogenemia|Acquired dysfibrinogenemia]]
* [[Fibrinogen#Congenital hypodysfibrinogenemia|Congenital hypodysfibrinogenemia]][[Fibrinogen#Cryofibrinogenemia|Cryofibrinogenemia]]
* [[Fibrinogen#Acquired hypofibrinogenemia|Acquired hypofibrinogenemia]]
|
* [[Epistaxis]]
* Easy [[Bruise|bruising]]
* [[Menorrhagia]]
* [[Muscle]] bleeds
* [[Hemarthrosis]]
* [[Bleeding]] from the [[umbilical cord]] stump after birth
* Bleeding after [[dental surgery]] or tooth extraction
* Abnormal bleeding during or after injury, surgery, or childbirth
* [[Gastrointestinal tract|Gastrointestinal]] [[hemorrhage]]
* [[Cerebral hemorrhage]]
* [[Thrombosis]]
|_
| +
| +
| +/-
| +
|N
|↑
|↑
|↑
|↑
|
* Impaired fibrin cross linking or clot dissolution.
* The severity of bleeding in patients with fibrinogen disorders can be mild or severe, with higher bleeding risk in those with afibrinogenemia or lower levels of functional fibrinogen.  The age of onset is also variable, with earlier onset in those with more severe deficiency.
|-
| colspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Prothrombin deficiency]]
|
* Easy [[bruising]]
* [[Epistaxis]]
* Soft-tissue hemorrhage
* Excessive postoperative bleeding
* [[Menorrhagia]]
* Muscle [[Hematoma|hematomas]]
* [[Hemarthrosis]]
* [[Intracranial hemorrhage|Intracranial]] bleeding
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|N
|N
|↑
|↑
|↑
|<nowiki>-</nowiki>
|-
| colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor V deficiency]]
|
* Excessive bruising with minor injuries
* [[Epistaxis]]
* [[Hemarthrosis]]
* [[Menorrhagia]]
* [[Intracerebral hemorrhage|Intracerebral hemorrhages]]
* [[Pulmonary hemorrhage]]
|_
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| +
| +
|N
|↑
|↑
|↑
|N
|The severity of bleeding is only partly related to the degree of factor V deficiency. Some patients with undetectable plasma levels of factor V experience only relatively mild bleeding.
|-
| colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor VII deficiency]]
|
* Easy [[Bruise|bruising]]
* Mucosal bleeding
* Postoperative bleeding
* [[Menorrhagia]]
* Soft tissue hematomas
* [[Thrombosis]]
|
|
|<nowiki>+</nowiki>
| +
| +
|N
|
|↑
|N
|N
|Thrombosis occurs in inherited factor VII deficiency  most cases are associated with the administration of factor VII replacement therapy
|-
| colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor X deficiency]]
|
* Prolonged bleeding following circumcision
* Easy [[Bruise|bruising]]
* [[Hematuria]]
* [[Menorrhagia]]
* Abortion
* Postpartum hemorrhage
* Epistaxis
* Pseudotumors
* Intracranial bleeding
* Hemarthroses
|<nowiki>+</nowiki>
| +
| +
| +
| +
|N
|N
|↑
|↑
|N
|<nowiki>-</nowiki>
|-
| colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor XII|Factor XII deficiency]]
|
* Majority,asymptomatic
* Recurrent miscarriages
* Painful leg ulcers
|_
|_
|_
|_
|_
|N
|N
|N
|↑
|N
|
|-
| colspan="3" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[High-molecular-weight kininogen|High molecular weight kininogen (HMWK)]] deficiency
|
* Possibility of positive family history of bleeding
|_
|_
|_
|_
|_
|N
|N
|N
|↑
|N
|
|-
| colspan="3" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Prekallikrein]] deficiency
|
* Possibility of positive family history of bleeding
|_
|_
|_
|_
|_
|N
|N
|N
|↑
|N
|
|-
| colspan="2" rowspan="1" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Factor XIII deficiency]]
| style="padding: 5px 5px; background: #DCDCDC;" |Types:
* Sub unit A mutation disease (more common)
* Sub unit B mutation disease
|
* Possibility of positive family history of bleeding
| -/+
|<nowiki>-/+</nowiki>
|<nowiki>-/+</nowiki>
|<nowiki>-/+</nowiki>
|<nowiki>-/+</nowiki>
|N
|N/
|N/↑
|N
|N
|
* Impaired fibrin cross linking or clot dissolution
* 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="pmid94489952">{{cite journal |vauthors=Aviña-Zubieta JA, Galindo-Rodriguez G, Lavalle C |title=Rheumatic manifestations of hematologic disorders |journal=Curr Opin Rheumatol |volume=10 |issue=1 |pages=86–90 |date=January 1998 |pmid=9448995 |doi= |url=}}</ref><ref name="pmid16551972">{{cite journal |vauthors=Plug I, Mauser-Bunschoten EP, Bröcker-Vriends AH, van Amstel HK, van der Bom JG, van Diemen-Homan JE, Willemse J, Rosendaal FR |title=Bleeding in carriers of hemophilia |journal=Blood |volume=108 |issue=1 |pages=52–6 |date=July 2006 |pmid=16551972 |doi=10.1182/blood-2005-09-3879 |url=}}</ref><ref name="pmid25059285">{{cite journal |vauthors=Blanchette VS, Key NS, Ljung LR, Manco-Johnson MJ, van den Berg HM, Srivastava A |title=Definitions in hemophilia: communication from the SSC of the ISTH |journal=J. Thromb. Haemost. |volume=12 |issue=11 |pages=1935–9 |date=November 2014 |pmid=25059285 |doi=10.1111/jth.12672 |url=}}</ref><ref name="pmid11307831">{{cite journal |vauthors=White GC, Rosendaal F, Aledort LM, Lusher JM, Rothschild C, Ingerslev J |title=Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis |journal=Thromb. Haemost. |volume=85 |issue=3 |pages=560 |date=March 2001 |pmid=11307831 |doi= |url=}}</ref><ref name="pmid24026910">{{cite journal |vauthors=Favaloro EJ, Meijer P, Jennings I, Sioufi J, Bonar RA, Kitchen DP, Kershaw G, Lippi G |title=Problems and solutions in laboratory testing for hemophilia |journal=Semin. Thromb. Hemost. |volume=39 |issue=7 |pages=816–33 |date=October 2013 |pmid=24026910 |doi=10.1055/s-0033-1356573 |url=}}</ref><ref name="pmid250592852">{{cite journal |vauthors=Blanchette VS, Key NS, Ljung LR, Manco-Johnson MJ, van den Berg HM, Srivastava A |title=Definitions in hemophilia: communication from the SSC of the ISTH |journal=J. Thromb. Haemost. |volume=12 |issue=11 |pages=1935–9 |date=November 2014 |pmid=25059285 |doi=10.1111/jth.12672 |url=}}</ref>
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Type A deficiency
|
* Eeasy [[Bruise|bruising]]
* Inadequate clotting in [[trauma]] or mild injury
* Spontaneous hemorrhage
* [[Hemarthrosis]]
* [[Epistaxis]]
* [[Gingival bleeding]]
|_
|_
| +
| +
|<nowiki>+</nowiki>
|N
|N
|N
|↑
|N
|<nowiki>-</nowiki>
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Type B deficiency
|
* Neonatal bleeding
* Trauma-related soft-tissue hemorrhage
* [[Hemarthrosis]] 
* [[Hematoma|Hematomas]]
|_
|_
|<nowiki>+</nowiki>
| +
|<nowiki>+</nowiki>
|N
|N
|N
|↑
|N
|<nowiki>-</nowiki>
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Type C deficiency
|
* Family history
* Bleeding after surgery or injury
|_
|_
|<nowiki>+</nowiki>
|Rare
|Rare
|N
|N
|N
|↑
|N
| -
|-
| rowspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |Rare diseases
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Disseminated intravascular coagulation|Disseminated Intravascular Coagulation]]
| style="padding: 5px 5px; background: #DCDCDC;" |
* [[Trauma]]
* Burn
* [[Crush injury]]
* [[Sepsis]]
* [[Malignancy]]
* Obstetric complication: abruption, amniotic fluid embolism
* [[Hemolytic anemia]]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|_
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|↓
|↑
|↑
|↑
|N
| -
|-
| colspan="2" align="center" style="padding: 5px 5px; background: #DCDCDC;" |[[Vitamin K Deficiency]]
| style="padding: 5px 5px; background: #DCDCDC;" |
* Bleeding after trauma
* [[Epistaxis]]
* [[Hematoma]]
* Gastrointestinal bleeding
* [[Menorrhagia]]
* [[Hematuria]]
* Gum bleeding
* Oozing from venipuncture sites
* Easy [[Bruise|bruisability]]
|<nowiki>+</nowiki>
| +
| +
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|N
|↑
|↑
|Normal or mildly prolonged
|N
| -
|}
{| class="wikitable"
|+
Different types of Von-Willebrand diseases can be differentiated from each other based on the following table:<ref name="pmid258585643">{{cite journal |vauthors=Bodó I, Eikenboom J, Montgomery R, Patzke J, Schneppenheim R, Di Paola J |title=Platelet-dependent von Willebrand factor activity. Nomenclature and methodology: communication from the SSC of the ISTH |journal=J. Thromb. Haemost. |volume=13 |issue=7 |pages=1345–50 |date=July 2015 |pmid=25858564 |pmc=5576173 |doi=10.1111/jth.12964 |url=}}</ref>
!Type of VWD
!
!Type of factor deficiency
!Prevalence
!Inheritance pattern
!Clinical manifestations
!VWF activity
!RIPA
!Factor VIII
|-
|Type 1
|
|Quantitative/ partial
|60-70%
|AD
|
* Bleeding severity mild to severe
|↓
|↓
|↓
|-
| rowspan="4" |Type 2
|2A<ref name="pmid1537829">{{cite journal |vauthors=Lyons SE, Bruck ME, Bowie EJ, Ginsburg D |title=Impaired intracellular transport produced by a subset of type IIA von Willebrand disease mutations |journal=J. Biol. Chem. |volume=267 |issue=7 |pages=4424–30 |date=March 1992 |pmid=1537829 |doi= |url=}}</ref>
|Qualitative
|10%
|AD/AR
|
* Moderate to severe bleeding
|↓
|↓
|N or ↓
|-
|2B
|Qualitative
|5%
|AD
|
* [[Thrombocytopenia]]
* Moderate to severe bleeding
|↓
|↑
|N or↓
|-
|2M
|Qualitative
|<1%
|AD/AR
|
* Moderate to severe bleeding
|↓
|↓
|N or ↓
|-
|2N
|Qualitative
|<1%
|AR
|
* Clinically similar to [[hemophilia A]] with joint, soft tissue, urinary bleeding
|N
|N
|↓
|-
|Type 3
|
|Complete deficiency
|1-2%
|AR
|
* Clinically similar to [[hemophilia A]] with joint and soft tissue bleeding
* Severe [[mucosal bleeding]]
|Absent
|↓
|
Low, 1-10%
|}
For more information on Von Willebrand disease [[ von willebrand disease | click here]]


{| class="wikitable"
{| class="wikitable"

Revision as of 11:28, 20 September 2018

Autoimmune hemolytic anemia Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assosciate Editor(s)-In-Chief: Prashanth Saddala M.B.B.S; Shyam Patel [2], Irfan Dotani [3]

Overview

A variety of conditions comprise the differential diagnosis of autoimmune hemolytic anemia. These include microangiopathic hemolytic anemia, paroxysmal cold hemoglobinuria, paroxysmal nocturnal hemoglobinuria, hereditary spherocytosis, pernicious anemia, and chronic lymphocytic leukemia. The diagnosis of autoimmune hemolytic anemia can sometimes be made by first ruling out these other causes. It is important to distinguish amongst these conditions since the prognosis and treatment of each condition is different.

Differentiating Autoimmune hemolytic anemia from other Diseases

Characteristic Causes Pathophysiology Laboratory abnormalities Physical examination Therapy Other associations
Autoimmune hemolytic anemia
  • Polyclonal antibody production that binds to and targets red blood cells for destruction intravascurly or extravascularly[1]
  • Removal of offending agent
  • Corticosteroids
  • Cyclophosphamide
  • Cyclosporine A
  • Azathioprine
  • Rituximab
  • Splenectomy
  • Hemolysis can occur at warm or cold temperatures
Microangiopathic hemolytic anemia
  • Formation of small vessel microthombi
  • Bleeding
  • Thrombosis
  • Pyrexia
  • Altered mental status
  • Neurologic deficits
  • Impaired urine output
  • Can be life-threatening pending the underlying cause
  • TTP required immediate treatment
Paroxysmal cold hemoglobinuria
  • Biphasic hemolysin (IgG) that binds red blood cells and low temperatures and triggers complement-mediated intravascular hemolysis at warm temperatures[2]
  • Associated with syphilis[2]
  • Maternal IgG can cross the placenta and affect the fetus[2]
Paroxysmal nocturnal hemoglobinuria
  • Genetic defect in anchoring proteins for complement factors on red blood cells
  • Hemolysis due to loss of complement inhibition on the red blood cell surface, which in turn is due to defect in CD55 (decay accelerating factor) and CD59 (membrane inhibitor of reactive lysis) or other glycophosphatidylinositol-anchored proteins on the red blood cell membrane
  • Absence of CD55 and CD59 by flow cytometry
  • Splenomegaly
  • Abdominal tenderness
  • Pallor
Hereditary spherocytosis[3]
  • Mutation in ankyrin[3]
  • Mutation in alpha- or beta-spectrin[3]
  • Mutation in band 3[3]
  • Mutation in protein 4.2[3]
  • Positive eosin-5-maleimide binding to red blood cells[3]
  • Positive osmotic fragility testing[3]
  • Spherocytes on peripheral blood smear
  • Can be autosomal dominant or recessive
Pernicious anemia[4]
  • Autoimmune gastritis[4]
  • Production of anti-intrinsic factor antibodies[4]
  • Production of anti-parietal cell antibodies[4]
  • Impaired vitamin B12 absorption due to absence of intrinsic factor
  • Low vitamin B12 level
  • Presence of anti-intrinsic factor antibodies
  • Presence of anti-parietal cell antibodies
  • Associated with diabetes, thyroid disease, vitiligo and other autoimmune conditions
Chronic lymphocytic leukemia[5]
  • Mutations in hematopoietic stem cells and B lymphocytes
  • Clonal proliferation of malignant B lymphocytes
  • Elevated absolute lymphocyte count
  • Anemia (Rai stage III) and thrombocytopenia (Rai stage IV)
  • Chemotherapy with rituximab
  • Ibrutinib
  • Venetoclax
  • Secondary autoimmune hemolytic anemia occurs in 10-25% of patients with CLL
  • Treatment with corticosteroids or anti-leukemic therapy will correct the underlying anemia

References

  1. ↑ 1.0 1.1 1.2 Berentsen S, Sundic T (2015). "Red blood cell destruction in autoimmune hemolytic anemia: role of complement and potential new targets for therapy". Biomed Res Int. 2015: 363278. doi:10.1155/2015/363278. PMC 4326213. PMID 25705656.
  2. ↑ 2.0 2.1 2.2 2.3 Akpoguma AO, Carlisle TL, Lentz SR (2015). "Case report: paroxysmal cold hemoglobinuria presenting during pregnancy". BMC Hematol. 15: 3. doi:10.1186/s12878-015-0023-7. PMC 4334594. PMID 25699184.
  3. ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Gallagher PG (2013). "Abnormalities of the erythrocyte membrane". Pediatr Clin North Am. 60 (6): 1349–62. doi:10.1016/j.pcl.2013.09.001. PMC 4155395. PMID 24237975.
  4. ↑ 4.0 4.1 4.2 4.3 4.4 Chan CQ, Low LL, Lee KH (2016). "Oral Vitamin B12 Replacement for the Treatment of Pernicious Anemia". Front Med (Lausanne). 3: 38. doi:10.3389/fmed.2016.00038. PMC 4993789. PMID 27602354.
  5. ↑ Kipps TJ, Stevenson FK, Wu CJ, Croce CM, Packham G, Wierda WG; et al. (2017). "Chronic lymphocytic leukaemia". Nat Rev Dis Primers. 3: 16096. doi:10.1038/nrdp.2016.96. PMC 5336551. PMID 28102226.


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