Thrombotic Thrombocytopaenic Purpura/Haemolytic Uraemic Syndrome
Description Diagnostic criteria Medicine treatment Referral ICD-10 code: M31.1/D59.3 DESCRIPTION An acute syndrome that presents with diarrhoea/dysentery (although diarrhoea-negative cases are increasingly recognised) with varying combinations of the following: Non-immune microangiopathic haemolytic anaemia with fragmentation haemolysis (schistocytes), thrombocytopenia often with purpura, a
Description
Diagnostic criteria
Medicine treatment
Referral
ICD-10 code: M31.1/D59.3
DESCRIPTION
An acute syndrome that presents with diarrhoea/dysentery (although diarrhoea-negative cases are increasingly recognised) with varying combinations of the following: Non-immune microangiopathic haemolytic anaemia with fragmentation haemolysis (schistocytes), thrombocytopenia often with purpura, acute kidney injury, fever and neurologic abnormalities.
DIAGNOSTIC CRITERIA
Investigations
- HIV testing
- INR and PTT are normal as compared to DIC where they are abnormal.
- Stool for Shiga toxin producing E coli.
- Blood cultures.
- FBC and smear.
- Urea and electrolytes.
MEDICINE TREATMENT
Specialist initiated
- Fresh frozen plasma (FFP), IV, 30 mL/kg/day over 20-30 minutes in 3– 4 doses.
OR
- Freeze dried plasma (FDP), IV, 30 mL/kg/day over 20-30 minutes in 3–4 doses.
For Infections
- Ceftriaxone, IV, 50–80 mg/kg once daily.
Need for platelet transfusion to be discussed with a haematologist. Early dialysis – discuss with a nephrologist.
REFERRAL
All patients – early consultation and transfer to a regional centre.
Standard Treatment Guidelines and Essential Medicines List for South Africa Paediatric Hospital_2023 edition_ Updated February 2025
Chapter 3: Blood and Blood Forming Organs, 3.10 Thrombotic Thrombocytopaenic Purpura/Haemolytic Uraemic Syndrome