THROMBOTIC THROMBOCYTOPENIC PURPURA-HAEMOLYTIC URAEMIC SYNDROME (TTP-HUS)
Acute syndromes with abnormalities in multiple organ systems with evidence of micro-angiopathic haemolytic anaemia and thrombocytopenia. This condition presents with:
» thrombocytopenia, often with purpura but not usually severe bleeding,
» acute renal insufficiency that may be associated with anuria and may require acute dialysis, » neurologic abnormalities, and » fever.
TTP-HUS is associated with HIV infection and patients should be tested for HIV. TTP-HUS should be distinguished from disseminated intravascular coagulation (DIC) and severe pre-eclampsia where the coagulation profile is deranged.
In HIV-associated thrombotic thrombocytopenia, start combination antiretroviral therapy urgently.
- Fresh frozen plasma, IV infusion, 30 mL/kg in 3–4 divided doses.
The use of platelet transfusions should be discussed with a specialist.
» All patients – discuss with a haematologist.