Bleeding diathesis: Difference between revisions

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! rowspan="2" |Category
! rowspan="2" |Category
! rowspan="2" |Sub-category
! rowspan="2" |Sub-category
! rowspan="2" |Diseases
! colspan="2" rowspan="2" |Diseases
! rowspan="2" |History
! rowspan="2" |History
! colspan="5" |Clinical manifestation
! colspan="5" |Clinical manifestation
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| rowspan="13" |[[Platelet]] disorders
| rowspan="13" |[[Platelet]] disorders
| rowspan="7" |[[Thrombocytopenia]]
| rowspan="7" |[[Thrombocytopenia]]
|[[Infection]]-Induced Thrombocytopenia<span name="harr_c115s002s001s002p001"></span><span name="9100754"></span>
| colspan="2" |[[Infection]]-Induced Thrombocytopenia<span name="harr_c115s002s001s002p001"></span><span name="9100754"></span>
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* History of prior infection
* History of prior infection
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|[[Medication|Medications]]-Induced Thrombocy<span name="harr_c115s002s001s003p001"></span><span name="9100757"></span>topenia
| colspan="2" |[[Medication|Medications]]-Induced Thrombocy<span name="harr_c115s002s001s003p001"></span><span name="9100757"></span>topenia
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* History of [[Medication|medications]]
* History of [[Medication|medications]]
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|[[Heparin]]-Induced Thrombocytopenia<span name="harr_c115s002s001s004p001"></span><span name="9100761"></span>
| colspan="2" |[[Heparin]]-Induced Thrombocytopenia<span name="harr_c115s002s001s004p001"></span><span name="9100761"></span>
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* [[Thrombosis]]
* [[Thrombosis]]
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|[[Idiopathic thrombocytopenic purpura|Immune Thrombocytopenic Purpura]] ([[Idiopathic thrombocytopenic purpura|ITP]])<span name="harr_c115s002s001s005p001"></span><span name="9100771"></span>
| colspan="2" |[[Idiopathic thrombocytopenic purpura|Immune Thrombocytopenic Purpura]] ([[Idiopathic thrombocytopenic purpura|ITP]])<span name="harr_c115s002s001s005p001"></span><span name="9100771"></span>
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* Prior [[infection]] or no history
* Prior [[infection]] or no history
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|Inherited [[Thrombocytopenia]]<span name="harr_c115s002s001s006p001"></span><span name="9100783"></span>
| colspan="2" |Inherited [[Thrombocytopenia]]<span name="harr_c115s002s001s006p001"></span><span name="9100783"></span>
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* Family history
* Family history
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|[[Thrombotic thrombocytopenic purpura|Thrombotic Thrombocytopenic Purpura]] ([[Thrombotic thrombocytopenic purpura|TTP]])<span name="harr_c115s002s002s001p001"></span><span name="9100787"></span>
| colspan="2" |[[Thrombotic thrombocytopenic purpura|Thrombotic Thrombocytopenic Purpura]] ([[Thrombotic thrombocytopenic purpura|TTP]])<span name="harr_c115s002s002s001p001"></span><span name="9100787"></span>
|History of:
|History of:
*[[Cancer]]
*[[Cancer]]
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|[[Hemolytic-uremic syndrome|Hemolytic Uremic Syndrome]]<span name="harr_c115s002s002s002p001"></span><span name="9100796"></span>
| colspan="2" |[[Hemolytic-uremic syndrome|Hemolytic Uremic Syndrome]]<span name="harr_c115s002s002s002p001"></span><span name="9100796"></span>
|History of:
|History of:
* Infections
* Infections
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| colspan="2" |Thromobcytosis<span name="harr_c115s002s003p001"></span><span name="9100800"></span>
| colspan="3" |Thromobcytosis<span name="harr_c115s002s003p001"></span><span name="9100800"></span>
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* Digital pain
* Digital pain
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| rowspan="5" |Qualitative Disorders of [[Platelet]] Function<span name="harr_c115s002s004s001p001"></span><span name="9100803"></span>
| rowspan="5" |Qualitative Disorders of [[Platelet]] Function<span name="harr_c115s002s004s001p001"></span><span name="9100803"></span>
| rowspan="2" |Inherited Disorders of [[Platelet]] Function<span name="harr_c115s002s004s001p001"></span><span name="9100803"></span>
| rowspan="3" |Inherited Disorders of [[Platelet]] Function<span name="harr_c115s002s004s001p001"></span><span name="9100803"></span>
|Glanzmann’s thrombasthenia
|Glanzmann’s thrombasthenia
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|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
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|Bernard-Soulier syndrome
|Bernard-Soulier syndrome
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|AR inheritance/ absence of the platelet Gp Ib-IX-V receptor
|AR inheritance/ absence of the platelet Gp Ib-IX-V receptor
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|Platelet storage pool disorder (SPD)
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|Platelet storage pool disorder (SPD)
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|Acquired Disorders of [[Platelet]] Function<span name="harr_c115s002s004s002p001"></span><span name="9100808"></span>
|Acquired Disorders of [[Platelet]] Function<span name="harr_c115s002s004s002p001"></span><span name="9100808"></span>
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|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
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|[[Von Willebrand disease|Von Willebrand Disease]]<span name="harr_c115s002s005p001"></span><span name="9100810"></span>
|[[Von Willebrand disease|Von Willebrand Disease]]<span name="harr_c115s002s005p001"></span><span name="9100810"></span>
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* Easy bruising
* Easy bruising
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| rowspan="2" |Vessel wall disorders
| rowspan="2" |Vessel wall disorders
| colspan="2" |[[Metabolism|Metabolic]] and [[Inflammation|Inflammatory]] Disorders
| colspan="2" |[[Metabolism|Metabolic]] and [[Inflammation|Inflammatory]] Disorders
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| colspan="2" |Inherited Disorders of the [[Vessel wall|Vessel Wall]]
| colspan="2" |Inherited Disorders of the [[Vessel wall|Vessel Wall]]
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| rowspan="12" |Coagulation factor disorders
| rowspan="12" |Coagulation factor disorders
| colspan="2" |[[Fibrinogen]] deficiency
| colspan="2" |[[Fibrinogen]] deficiency
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* [[Epistaxis]]
* [[Epistaxis]]
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| colspan="2" |[[Prothrombin deficiency]]
| colspan="2" |[[Prothrombin deficiency]]
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* Easy [[bruising]]
* Easy [[bruising]]
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| colspan="2" rowspan="1" |[[Factor V deficiency]]
| colspan="2" rowspan="1" |[[Factor V deficiency]]
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* Excessive bruising with minor injuries
* Excessive bruising with minor injuries
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| colspan="2" rowspan="1" |[[Factor VII deficiency]]
| colspan="2" rowspan="1" |[[Factor VII deficiency]]
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* Easy [[Bruise|bruising]]
* Easy [[Bruise|bruising]]
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| colspan="2" rowspan="1" |[[Factor X deficiency]]
| colspan="2" rowspan="1" |[[Factor X deficiency]]
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* Prolonged bleeding following circumcision
* Prolonged bleeding following circumcision
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| colspan="2" rowspan="1" |[[Factor XII|Factor XII deficiency]]
| colspan="2" rowspan="1" |[[Factor XII|Factor XII deficiency]]
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* Majority,asymptomatic
* Majority,asymptomatic
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| colspan="2" |HK deficiency
| colspan="2" |HK deficiency
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| colspan="2" rowspan="1" |[[Prekallikrein]] deficiency
| colspan="2" rowspan="1" |[[Prekallikrein]] deficiency
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| colspan="2" rowspan="1" |[[Factor XIII deficiency]]
| colspan="2" rowspan="1" |[[Factor XIII deficiency]]
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| rowspan="3" |[[Hemophilia]]
| rowspan="3" |[[Hemophilia]]
|Type A deficiency
|Type A deficiency
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* Eeasy bruising
* Eeasy bruising
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|Type B deficiency
|Type B deficiency
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* Neonatal bleeding
* Neonatal bleeding
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|Type C deficiency
|Type C deficiency
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* Family history of bleeding disorder,
* Family history of bleeding disorder,
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| rowspan="4" |Rare diseases
| rowspan="4" |Rare diseases
| colspan="2" |[[Disseminated intravascular coagulation|Disseminated Intravascular Coagulation]]
| colspan="2" |[[Disseminated intravascular coagulation|Disseminated Intravascular Coagulation]]
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* Trauma
* Trauma
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| colspan="2" |[[Vitamin K Deficiency]]
| colspan="2" |[[Vitamin K Deficiency]]
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* Bleeding after trauma
* Bleeding after trauma
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| colspan="2" |Coagulation Disorders Associated with [[Hepatic failure|Liver Failure]]
| colspan="2" |Coagulation Disorders Associated with [[Hepatic failure|Liver Failure]]
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| colspan="2" |Acquired Inhibitors of Coagulation Factors
| colspan="2" |Acquired Inhibitors of Coagulation Factors
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Revision as of 16:34, 10 September 2018


Bleeding diathesis main page

Overview

Classification

Differential Diagnosis

Platelet disorders
Immune Thrombocytopenic Purpura
Thrombotic Thrombocytopenic Purpura
Hemolytic Uremic Syndrome
Thrombocytosis
Von Willebrand Disease
Coagulation disorders
Fibrinogen deficiency
Prothrombin deficiency
Factor V deficiency
Factor VII deficiency
Factor VIII deficiency
Factor IX deficiency
Factor X deficiency
Factor XI deficiency
Factor XII deficiency
High-molecular-weight kininogen deficiency
Prekallikrein deficiency
Factor XIII deficiency
Hemophilia
Rare diseases
Disseminated Intravascular Coagulation
Vitamin K Deficiency

Different types of Von-Willebrand diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Mehrian Jafarizade, M.D [2], Nazia Fuad M.D.

Overview

Bleeding diathesis is unusual susceptibility to bleed due to hypo-coagulopathies. 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, prothrombin time, activated partial thromboplastin time, thrombin time and reptilase time.

Homostasis

Hemostasis is the process of blood clot formation at the site of bleeding. This process has 4 main phases as below:

Platelet plug formation

Coagulation cascade

Antithrombotic control

Fibrinolysis

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:

Platelet disorders:

  1. Thrombocytopenia: platelet count less than 150,000 per mm3
  2. Thromobcytosis: platelet countcmore than 450,000 per mm3
  3. Qualitative Disorders of Platelet function such as Von Willebrand Disease, inherited or acquired functional disorders.

Coagulation disorders

  1. Vessel wall disorders: Endothelial cells are lining entire vessel walls all over the body. Endothelium is an active layer responsible for inflammatory responses, angiogenesis and blood cell interactions. Endothelial cells have a very important role in hemostasis and they are regulating blood fluidity by the balance of antithrombotic/prothrombotic and vasodilatory/vasoconstrictor effects.
  2. Coagulation factor disorders:
  3. Disseminated Intravascular Coagulation
  4. Vitamin K Deficiency
  5. Coagulation Disorders Associated with Liver Failure
  6. Acquired Inhibitors of Coagulation Factors

Differential Diagnosis

Differential diagnosis of "Bleeding disorders":
Category Sub-category Diseases History Clinical manifestation Laboratory testing Comments
Petechiae Ecchymoses Menorrhagia Hematoma Hemarthrosis Platelet count Bleeding time (BT) Prothrombin time (PT) Activated partial thromboplastin time (aPTT) Thrombin time (TT)
Platelet disorders Thrombocytopenia Infection-Induced Thrombocytopenia
  • History of prior infection
+ + + + + N N
Medications-Induced Thrombocytopenia + + + + + N N
Heparin-Induced Thrombocytopenia + + + + +
Immune Thrombocytopenic Purpura (ITP) + + + + + N N
Inherited Thrombocytopenia
  • Family history
+ + + + + N N
Thrombotic Thrombocytopenic Purpura (TTP) History of: + + + + +
Hemolytic Uremic Syndrome History of:
  • Infections
+ + + + + N N
Thromobcytosis + + Normal or slightly prolonged ɴ ɴ
Qualitative Disorders of Platelet Function Inherited Disorders of Platelet Function Glanzmann’s thrombasthenia + + + + + AR inheritance/ absence of the platelet Gp IIb/IIIa receptor
Bernard-Soulier syndrome AR inheritance/ absence of the platelet Gp Ib-IX-V receptor
Platelet storage pool disorder (SPD) AD inheritance/ abnormalities of platelet granule formation
Acquired Disorders of Platelet Function + + + + +
Von Willebrand Disease + + + + + Ν
Vessel wall disorders Metabolic and Inflammatory Disorders
Inherited Disorders of the Vessel Wall
Coagulation factor disorders Fibrinogen deficiency _ + + + N 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.
Prothrombin deficiency + + + + + N N
Factor V deficiency _ + + + + 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.
Factor VII deficiency + + + N N Thrombosis occurs in inherited factor VII deficiency most cases are associated with the administration of factor VII replacement therapy
Factor X deficiency
  • Prolonged bleeding following circumcision
+ + + + + N N N
Factor XII deficiency
  • Majority,asymptomatic
  • Recurrent miscarriages
  • Painful leg ulcers
_ _ _ _ _ N N N
HK deficiency
Prekallikrein deficiency
Factor XIII deficiency
Hemophilia Type A deficiency
  • Eeasy bruising
  • Inadequate clotting in trauma or mild injury
  • Spontaneous hemorrhage.
  • Hemarthrosis
  • Epistaxis
  • Gingival bleeding
_ _ + + + Unaffected Unaffected Unaffected Prolonged N
Type B deficiency
  • Neonatal bleeding
  • Trauma-related soft-tissue hemorrhage
  • Hemarthrosis
  • Hematomas
_ _ + + + Unaffected N N N
Type C deficiency
  • Family history of bleeding disorder,
  • Bleeding after surgry or injury
_ _ + Rare Rare N N N N
Rare diseases Disseminated Intravascular Coagulation
  • Trauma
  • Burn
  • Crush injury
  • Sepsis
  • Malignancy
  • Obstetric complication: abruption, amniotic fluid embolism
  • Hemolytic anemia
+ + _ + +
Vitamin K Deficiency
  • Bleeding after trauma
  • Epistaxis
  • Hematoma
  • Gastrointestinal bleeding,
  • Menorrhagia
  • Hematuria
  • Gum bleeding
  • Oozing from venipuncture sites
  • Easy bruisability
+ + + + + N Normal or mildly prolonged
Coagulation Disorders Associated with Liver Failure
Acquired Inhibitors of Coagulation Factors
Different types of Von willebrand diseases can be differentiate from each other based on below table:
Type of VWD Type of factor deficiency Prevalence Inheritance pattern Clinical manifestations VWF activity RIPA Factor VIII Plt levels
Type 1 Quantitative/ partial
Type 2 2A
2B
2M
2N
Type 3