Thrombotic Thrombocytopenic Purpura-Haemolytic Uraemic Syndrome (TTP-HUS)
Description Medicine Treatment Referral D59.3 + (M31.1) DESCRIPTION Acute syndromes with abnormalities in multiple organ systems and 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 and thrombocytopenia, often with purpura but not usuall
D59.3 + (M31.1)
DESCRIPTION
- Acute syndromes with abnormalities in multiple organ systems and 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 and thrombocytopenia, often with purpura but not usually severe bleeding,
- acute renal insufficiency,
- neurologic abnormalities, and/or
- fever.
Note:
The presence of fragments and low platelets is enough to consider the diagnosis.
- Microangiopathic haemolytic anaemia is defined as non-immune haemolysis with prominent RBC fragmentation (schistocytes) observed on the peripheral blood smear along with thrombocytopaenia.
- TTP-HUS is often 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, in the latter, the coagulation profile (PT/PTT) is also deranged.
MEDICINE TREATMENT
- This is a medical emergency.
- In HIV-associated thrombotic thrombocytopenia, start combination antiretroviral therapy urgently.
- Platelet transfusions may be associated with increased morbidity and mortality. Use of platelet transfusions should be discussed with a specialist.
Transfusion of plasma products:
- Lyophilised plasma, IV infusion, 30 mL/kg/day in 3–4 divided doses.
OR
- Fresh frozen plasma, IV infusion, 30 mL/kg/day in 3–4 divided doses.
LoE:IIIbxxiv
REFERRAL
- All patients – discuss with a haematologist urgently.
Standard Treatment Guidelines And Essential Medicines List For South Africa, Adult Hospital level_Version 1_30 March 2026
Chapter 2: Blood and Blood Forming Organs; 2.6 Thrombotic Thrombocytopenic Purpura-Haemolytic Uraemic Syndrome (TTP-HUS)