DESCRIPTION
Anaemia due to destruction of red blood cells. Destruction may be due to:
» Extracellular factors such as auto-immunity or mechanical factors, e.g.
disseminated intravascular coagulation (DIC), hypersplenism,
medications.
» Abnormalities of the cell membrane, e.g. hereditary spherocytosis.
» Enzymes, e.g. G6PD deficiency.
» Haemoglobin, e.g. sickle cell anaemia, thalassaemia.
Investigations
Evidence of haemolysis: anaemia, reticulocytosis, decreased haptoglobin,
increased lactate dehydrogenase (LDH) and unconjugated
hyperbilirubinaemia.
Coombs’ test (direct antiglobulin) is usually positive with autoimmune haemolysis.
GENERAL MEASURES
Treat the underlying cause.
Do not transfuse prior to appropriate investigations, unless anaemia is severe.
Coombs-positive haemolytic anaemia may be technically difficult to cross match.
Efficacy of transfusion is limited by the shortened red cell survival due to haemolysis.
In G6PD deficiency, avoid drugs known to cause haemolysis, including aspirin, sulphonamides (including cotrimoxazole), dapsone and primaquine. In patients with cold agglutinins all transfusions must be given through a
blood warmer to avoid cold-induced haemolysis.
MEDICINE TREATMENT
All patients:
Because of high red cell turnover, supplement with:
Folic acid, oral, 5 mg daily.
Autoimmune haemolytic anaemia
Treat under specialist supervision.
Prednisone, oral, 1–2 mg/kg daily, initial dose.
o When a satisfactory response is obtained with recovery of the
haemoglobin and a decrease in LDH serum concentrations, taper
dose over a period of 4 weeks to 30 mg daily.
o Thereafter further reduction should be slower to prevent disease
recurrence.
Prednisone treatment can be stopped when the Coombs’ reaction
becomes negative.
If inadequate response:
ADD
Azathioprine, oral, 2.5 mg/kg daily.
o Titrate to Hb response.
o May be required for several months
o Monitor for neutropenia.
Patients who fail medicine treatment should be considered for splenectomy.
REFERRAL/CONSULTATION
» No response to medicine treatment.
» Other causes of haemolytic anaemia.
Reference and further reading
Standard Treatment Guidelines and Essential Medicines List for South Africa 2012 Edition
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