Neobook — clinical reference

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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, mechanical heart valves.
Abnormalities of the cell membrane, e.g. hereditary spherocytosis.
Enzymes, e.g. G6PD deficiency.
Haemoglobin abnormalities, e.g. sickle cell anaemia, thalassaemia.
Thrombotic thrombocytopaenic purpura: TTP is a life-threatening emergency. Refer immediately to a specialist unit for plasma infusion or exchange (see: Thrombotic thrombocytopenic purpura-Haemolytic uraemic syndrome).

Investigations

Evidence of haemolysis: anaemia, reticulocytosis, decreased haptoglobin, increased lactate dehydrogenase (LDH) and unconjugated hyperbilirubinaemia.
FBC and peripheral smear: spherocytes often reported
Coombs’ test (direct antiglobulin) is usually positive with autoimmune haemolysis.
HIV status.

Treat the underlying cause.
Do not transfuse prior to conducting appropriate investigations unless anaemia requires immediate intervention.
In situations of life-threatening anaemia, transfuse the most compatible unit of red blood cells and get specialist advice urgently. 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, such as aspirin, sulphonamides (including co-trimoxazole), dapsone, methylene blue, and primaquine.
In patients with cold agglutinins, all transfusions must be given through a blood warmer to avoid cold-induced haemolysis.

Because of high red cell turnover, supplement with:

Autoimmune haemolytic anaemia

Treat under specialist supervision.

Prednisone, oral.
- Initial dose: 1 mg/kg daily, until Hb is stable and >10 g/dL.
- Taper slowly and monitor Hb at least once weekly. (Refer to Prescribing Information for Specific Medicines for an example of a dose reduction regimen).
- Glucocorticoids should be tapered slowly upon normalization of the haemoglobin and LDH. The patient should be monitored for recurrence following cessation of treatment.
- As these conditions can often be life-threatening, specialist advice should be sought as early as possible after diagnosis.

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Indications of inadequate response:

Haemolysis remains severe after 3 weeks of prednisone dosed at 1 mg/kg.
Remission cannot be maintained on low doses of prednisone.
The patient has intolerable adverse effects or contraindications to glucocorticoids.

Refer to specialist for second-line treatment:

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Standard Treatment Guidelines And Essential Medicines List For South Africa, Adult Hospital level, 2020-4_Version 1_30 March 2026

Chapter 2: Blood and Blood Forming Organs; 2.1.4 Anaemia, Haemolytic

Anaemia, Haemolytic