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Appendix 102.1

ElementComment
Causes

Sepsis

Trauma (major injury, head injury, fat embolism)

Malignancy (e.g. acute myeloid leukaemia)

Obstetric complication (amniotic fluid embolism, placental abruption)

Immune-mediated disorder (e.g. anaphylaxis, haemolytic transfusion reaction, transplant rejection)

Other causes include giant haemangioma, abdominal aortic aneurysm, snake venom, amphetamine poisoning, cardiac arrest, drowning and heat stroke

Clinical features

Bleeding from skin and mucosae (nose and gums)

Bleeding from surgical incisions, wounds, venepuncture sites

Acute kidney injury

Jaundice Acute respiratory distress syndrome (ARDS) due to diffuse alveolar haemorrhage

Delirium, seizures

Adrenal insufficiency (adrenal haemorrhage)

Purpura fulminans

Full blood count and film

Thrombocytopenia

Fragmented red cells (schistocytes) in 50%

Blood results

Prolonged prothrombin and activated partial thromboplastin times

Low fibrinogen concentration

Raised concentration of fibrin degradation products/D-dimer

Differential diagnosis

Thrombotic thrombocytopenic purpura (Appendix 102.2)

Chronic DIC (Trousseau syndrome)

Acute liver failure

Decompensated chronic liver disease

HELLP syndrome of pregnancy (haemolysis, elevated liver enzymes, low platelet count) (see Table 100.8)

Amyloidosis

Management

Seek urgent advice from a haematologist

Treatment is of the underlying disorder

Consider blood product replacement therapy if the patient is actively bleeding or requires an intervention