Hereditary Spherocytosis

Exam Cram

• Hereditary Spherocytosis: 80% autosomal dominant

• Investigations: anaemia, spherocytes on blood film

• Diagnosis: cryo-haemolysis & EMA binding  (eosin-5-maleimide-binding tests)

• Management: splenectomy, no role for steroids

Notes

O/E: splenomegaly

Associated with increased risks of gallstone (black pigment), managed by cholecystectomy