Anaemia, Haemolytic
Description Diagnostic criteria General and supportive measures Medicine treatment Surgical treatment Referral ICD-10 code: D55–59 DESCRIPTION Anaemia caused by excessive destruction of red blood cells. Destruction may be due to: Corpuscular defects: abnormalities of the cell membrane (e.g. hereditary spherocytosis), enzyme abnormalities (e.g. G6PD deficiency), or abnormal haemoglobin (e.g.
Description
Diagnostic criteria
General and supportive measures
Medicine treatment
Surgical treatment
Referral
ICD-10 code: D55–59
DESCRIPTION
Anaemia caused by excessive destruction of red blood cells.
Destruction may be due to:
- Corpuscular defects:
- abnormalities of the cell membrane (e.g. hereditary spherocytosis),
- enzyme abnormalities (e.g. G6PD deficiency), or
- abnormal haemoglobin (e.g. sickle cell anaemia, thalassaemia).
- Extra-corpuscular defects:
- Autoimmune or isoimmune: idiopathic warm- or cold-antibodies, infection-triggered, e.g. Mycoplasma pneumoniae, medicine-related, e.g. penicillin, secondary to autoimmune disorders, e.g. SLE, juvenile arthritis, secondary to tumours, e.g. lymphoma, thymoma.
- Non-immune: secondary to microangiopathy, e.g. haemolytic uraemic syndrome, infections causing haemolysis, e.g. malaria, miscellaneous causes, including hypersplenism.
DIAGNOSTIC CRITERIA
Clinical
- Pallor, jaundice, fatigue.
- Splenomegaly.
Investigations (before transfusion)
- Full blood count.
- Evidence of haemolysis:
- anaemia,
- decreased haptoglobin,
- reticulocytosis,
- unconjugated hyperbilirubinaemia,
- increased lactate dehydrogenase (LDH),
- urobilinogen in the urine.
- Direct Coombs test (direct antiglobulin) is positive with autoimmune haemolysis.
- Haemoglobin electrophoresis.
- Renal function is abnormal in haemolytic uraemic syndrome.
- Exclude other autoimmune disorders.
- Consider underlying neoplasms.
- In patients receiving recurrent transfusions (e.g. thalassaemia), monitor ferritin levels 3-monthly and discuss with referral centre if > 1000 mcg/L.
GENERAL AND SUPPORTIVE MEASURES
- After appropriate investigations, transfuse the patient and then discuss with paediatrician or paediatric haematologist.
- Coombs-positive autoimmune haemolytic anaemia may require transfusion with the least incompatible blood (if cross-matching yields no compatible units).
- In G6PD deficiency, avoid medicines known to cause haemolysis (e.g. aspirin, sulphonamides and primaquine) and be sure to give the patient a list of such medicines at discharge.
MEDICINE TREATMENT
Warm antibody autoimmune haemolytic anaemia
Under specialist supervision:
- Prednisone, oral, 2 mg/kg/day until a satisfactory response is obtained.
- Continue treatment for a minimum of 4 weeks.
- Taper dose slowly over several weeks while monitoring for relapse.
Chronic haemolytic anaemia
All patients indefinitely:
- Folic acid, oral, 2.5 mg daily between birth and 6 months and 5 mg daily for > 5 kg and/or 6 months to 5 years.
SURGICAL TREATMENT
Splenectomy for hereditary spherocytosis with Hb < 10 g/dL and transfusion dependent, but only after 5 years of age and following consultation with a paediatric haematologist.
Pre-splenectomy immunization
Two weeks prior to surgery
- Pneumococcus conjugate vaccine (PCV)-13, IM, 0.5 mL followed by pneumococcus polysaccharide (PPV)-23, IM, 0.5 mL 8 weeks later.
- Haemophilus influenzae, type B (Hib) booster, IM, 0.5 mL.
- Meningococcal conjugate vaccine (MCV), IM, 0.5 mL.
Post splenectomy
- Phenoxymethylpenicillin, oral, 12 hourly.
- If < 5 years: 125 mg.
- If > 5 years: 250 mg.
- Give until at least 18 years of age.
- Annual influenza vaccine, IM, 0.5 mL.
- After splenectomy:
- Pneumococcus conjugate vaccine (PCV)-13, IM, followed by pneumococcus polysaccharide (PPV)-23, IM (a month later).
- Haemophilus influenzae, type B (Hib) and meningococcal conjugate vaccine (MCV) booster.
For catch up of routine conjugate pneumococcal vaccination:
- <12 months of age: 3 dose series.
- 12 months of age and older: 2 doses 8 weeks apart. (See: Primary Healthcare Standard Treatment Guidelines, Immunisation).
REFERRAL
- Any child with haemolytic anaemia e.g. thalassaemia, especially those who are transfusion dependent (more than 10 transfusions) for assessment for chelation therapy.
- All cases associated with evidence of haemolysis as above should be managed in consultation with a paediatrician or paediatric haematologist.
Click here for 3.2.1 Thalassaemia
Click here for 3.2.2 Anaemia, Sickle Cell
Standard Treatment Guidelines and Essential Medicines List for South Africa Paediatric Hospital_2023 edition_Updated February 2025
Chapter 3: Blood and Blood-Forming Organs, 3.2 Anaemia, Haemolytic