Causes of clinical disorders

The three major mechanisms of systemic bleeding disorders are:

1. coagulation deficiencies (reduction or inhibition of circulatory coagulation factors)

2. platelet abnormalities: of platelet number or function

3. vascular defects: of vascular endothelium

A list of differential diagnoses of systemic bleeding disorders is presented in Table 35.1 . 1 Table 35.1 Differential diagnoses of systemic bleeding disorders

Vascular disorders

Inherited

(a) • hereditary haemorrhagic telangiectasia

• Marfan's syndrome

Acquired

• purpura simplex senile purpura Henoch-Schonlein purpura steroid purpura scurvy

Coagulation factor deficiency or inhibitor

Inherited

• haemophilia A

• haemophilia B

• von Willebrand's disease

Acquired

• disseminated intravascular coagulation (b) • liver disease

• vitamin K deficiency

• oral anticoagulant therapy or overdosage

Thrombocytopenia

Inherited

• amegakaryocytic thrombocytopenia

Acquired

• immune thrombocytopenic purpura

• drug-induced thrombocytopenia

• disseminated intravascular coagulation

(b) • bone marrow replacement or failure

• thrombotic thrombocytopenic purpura

• post-transfusion purpura

• splenic pooling

Functional platelet disorders

Inherited

• Glanzmann's thrombasthenia

• Bernard-Soulier syndrome

• storage pool deficiency

Acquired

• myeloproliferative disorders

• dysproteinaemias

Source: After Mitchell et al. 1 Adapted from Bleeding disorders, MIMS Disease Index 1996 with permission of MIMS Australia, a division of MediMedia Australia Pty Limited.

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