THROMBOTIC THROMBOCYTOPENIC PURPURA-HAEMOLYTIC URAEMIC SYNDROME (TTP-HUS)

DESCRIPTION

Acute syndromes with abnormalities in multiple organ systems with evidence of micro-angiopathic haemolytic anaemia and thrombocytopenia. This condition presents with:

»      anaemia,

»     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.

 

TREATMENT

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.

 

REFERRAL

»     All patients – discuss with a haematologist.