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Pathology
Abnormal red cell membrane resulting in spherocytes, with increased osmotic
fragility
Aetiology
Autosomal dominant inheritance
Signs
Anaemia, gallstones, splenomegaly, fluctuant jaundice
Symptoms
Often none, lethargy, abdominal pain
Investigations
Bloods:, FBC, reticulocyte count
Blood Film: Spherocytes and reticulocytes .Treatment
Medical: Folic acid, blood transfusion only if severely anaemic
Surgical: Splenectomy (not often required), cholecystectomy
Prognosis
Good
Cholecystitis
Key Facts
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Key Images
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Key References
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