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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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