Synopsis
Recognise membrane-related extravascular haemolysis, confirm the phenotype without over-interpreting a single screening assay, manage anaemia and gallstones, and plan splenectomy and infection prevention safely.
- Hereditary spherocytosis is an inherited red-cell membrane disorder causing poorly deformable spherocytes to be trapped and destroyed in the spleen.
- Anaemia, jaundice, splenomegaly, reticulocytosis and pigment gallstones vary from an incidental compensated phenotype to transfusion-dependent disease in infancy.
- A blood film may show dense spherocytes without central pallor, but autoimmune haemolysis also produces spherocytes and must be separated with a direct antiglobulin test.
Key red flags
A sudden profound anaemia with an unexpectedly low reticulocyte count after fever or rash requires urgent haematology assessment.
Investigation priorities
Quantify anaemia and marrow compensation.
Management branches
Anaemia, jaundice or splenomegaly occurs with spherocytes or a family history.
- Obtain blood count, reticulocytes, film, bilirubin, LDH and haptoglobin, documenting transfusion timing and the clinical family pedigree.
- Perform a direct antiglobulin test before attributing spherocytes to inheritance, then request eosin-5-maleimide testing through haematology when the phenotype remains compatible.
A person with stable disease develops abrupt pallor, breathlessness, fever or worsening jaundice.
Key medicines
Folic acidHaematology commonly prescribes 5 mg orally once daily when haemolysis is moderate or severe, during pregnancy or when dietary folate may not meet erythropoietic demand.
Phenoxymethylpenicillin after splenectomyA common adult regimen is 250 mg orally twice daily. Start after splenectomy, follow the current asplenia policy for duration and allergy alternatives, and continue lifelong when invasive pneumococcal risk remains high.