Thrombotic thrombocytopaenic purpura haemolytic uraemic syndrome

M31.1/D59.3


DESCRIPTION

An acute syndrome that presents with diarrhoea/dysentery or renal infection with varying combinations of the following:
Non-immune microangiopathic haemolytic anaemia with fragmentation haemolysis (schistocytes), thrombocytopaenia often with purpura, acute renal insufficiency, fever and neurologic abnormalities.

DIAGNOSTIC CRITERIA

Investigations

  • HIV testing
  • INR/PTT is normal as compared to DIC where it is abnormal.
  • Stool for Shiga toxin producing E coli.
  • Blood cultures.
  • FBC and smear.
  • Urea and electrolytes.

MEDICINE TREATMENT

  • Fresh frozen plasma (FFP) IV infusion, 30 mL/kg/day in 3-4 doses
    OR
  • Freeze dried plasma (FDP), IV infusion, 30 mL/kg/day in 3-4 doses

For Infections

  • Ceftriaxone, IV, 50–80 mg/kg once daily.
    OR

    If < 1 month old:
  • Cefotaxime, IV, 25–50 mg/kg/dose, 6 hourly.

Need for platelet transfusion to be discuss with a haematologist.
Early dialysis - discuss with a nephrologist.

REFERRAL

All patients - early consultation and transfer to a regional centre.