D59.3 + (M31.1)
DESCRIPTION
This is a medical emergency.
Acute syndromes with abnormalities in multiple organ systems with evidence of micro-angiopathic haemolytic anaemia and thrombocytopenia.
This condition presents with varying combinations of the following (only some of which may be present):
- Microangiopathic haemolytic anaemia thrombocytopenia, often with purpura but not usually severe bleeding,
- acute renal insufficiency,
- neurologic abnormalities, and
- fever.
Note: The presence of fragments and low platelets is enough to consider the diagnosis.
Microangiopathic haemolytic anaemia is defined as nonimmune haemolysis with prominent red blood cell (RBC) fragmentation (schistocytes) observed on the peripheral blood smear along with thrombocytopaenia.
TTP-HUS is associated with HIV infection and all patients should be tested for HIV.
TTP-HUS should be distinguished from disseminated intravascular coagulation (DIC) and severe pre-eclampsia where the coagulation profile (PT/PTT) is deranged.
TREATMENT
- In HIV-associated thrombotic thrombocytopenia, start combination antiretroviral therapy urgently.
- Lyophilised plasma, IV infusion, 30 mL/kg/day in 3–4 divided doses.
OR
FFP, IV infusion, 30 mL/kg/day in 3–4 divided doses.
Use of platelet transfusions should be discussed with a specialist.
REFERRAL
All patients – discuss with a haematologist urgently.