Haemolysis
Shortening of erythrocyte lifespan below the expected 120 days. Marked intravascular haemolysis may lead to jaundice and haemoglobinuria.
Causes
- Blood transfusion reactions.
- Malaria.
- Sickle cell haemolytic crisis.
- Drugs, e.g. high-dose penicillin, methyl dopa.
- Autoimmune (cold or warm antibody-mediated)—may be idiopathic or secondary, e.g. lymphoma, SLE, mycoplasma.
- Haemolytic uraemic syndrome/thrombotic thrombocytopaenic purpura (microangiopathic haemolytic anaemia).
- Trauma (cardiac valve prosthesis).
- Glucose-6-phosphate dehydrogenase deficiency—oxidative crises occur following ingestion of fava beans or administration of drugs (e.g. primaquine, sulphonamides) leading to rapid onset anaemia and jaundice.
Diagnosis
- Unconjugated hyperbilirubinaemia, increased urinary urobilinogen (increased RBC breakdown).
- Reticulocytosis (increased RBC production).
- Splenic hypertrophy (extravascular haemolysis).
- Methaemoglobinaemia, haemoglobinuria, free plasma haemoglobin (intravascular haemolysis), reduced serum haptoglobins.
- RBC fragmentation (microangiopathic haemolytic anaemia).
- Coombs’ test (immune-mediated haemolysis).
- Other (including haemoglobin electrophoresis, bone marrow biopsy).
Management
- Identification and specific treatment of the cause where possible.
- Blood transfusion to maintain haemoglobin >7g/dL.
- Massive intravascular haemolysis may lead to acute renal failure. Maintain a good diuresis and haemo(dia)filter if necessary.
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