Autoimmune HemolysisUrgent

Fatigue, jaundice, dark urine; Hb 62 g/L falling; ↑reticulocytes, ↑LDH, ↑indirect bilirubin, ↓haptoglobin; spherocytes on the film

• Hb falling >20 g/L a day
• Angina, breathlessness at rest, confusion
• Haemoglobinuria with AKI
• Thrombosis
• Low platelets as well (Evans syndrome)

• Hereditary spherocytosis (DAT negative)
• G6PD deficiency (bite cells)
• TTP/HUS/DIC (schistocytes)
• Delayed haemolytic transfusion reaction
• PNH (DAT negative)
• Malaria

• Admit: Hb <80 g/L, rapid fall, symptomatic anaemia or new diagnosis with active haemolysis
• ICU: Haemodynamic compromise, myocardial ischaemia, fulminant haemolysis
• Transfuse: Symptomatic or Hb <70 g/L (<80 g/L with heart disease) — never delayed for a fully compatible unit

• Warm AIHA responds to steroids in ~80% within 3 weeks, but most relapse on the taper — rituximab is the preferred second line; splenectomy is now third line.
• Give folic acid 5 mg daily while haemolysing; thromboprophylaxis for inpatients with active haemolysis.
• Long steroid courses (≥20 mg/day for >4 weeks, especially with rituximab): co-trimoxazole PJP prophylaxis, calcium + vitamin D, PPI if GI risk.
• Always look for a secondary cause — about half of AIHA is secondary (lymphoproliferative disease, SLE, infection, drugs).

• Withhold blood in life-threatening anaemia because the crossmatch is "incompatible"
• Expect steroids to work in cold agglutinin disease
• Start rituximab without hepatitis B screening
• Stop steroids abruptly

• Waiting for a compatible crossmatch in severe anaemia
• Missing drug-induced haemolysis
• Missing lymphoma, CLL or SLE behind "idiopathic" AIHA
• Steroids for cold agglutinin disease
• Stopping steroids too fast

Ward until Hb is stable; ICU for cardiac or neurological compromise. Haematology weekly during the taper and a search for a secondary cause.