Hereditary Spherocytosis Flashcards

1
Q

What is hereditary spherocytosis

A

RBCs are sphere shaped making them fragile and easily destroyed when passing through the spleen.

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2
Q

Inheritance of hereditary spherocytosis

A

Autosomal dominant

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3
Q

Presentation of hereditary spherocytosis

A

Jaundice, anaemia, gallstones, splenomegaly, episodes of haemolytic crisis, often triggered by infections, aplastic crisis

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4
Q

Diagnosis of hereditary spherocytosis

A

FHx, clinical features, along with spherocytes on blood film, MCHC raised on FBC.

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5
Q

Why are reticulocytes raised

A

Due to rapid turnover of RBCs

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6
Q

Management of herediary spherocytosis

A

Folate supplementation and splenectomy, removal of gallbladder if gallstones removed. Transfusions may be rerquired during acute crises

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7
Q

What is hereditary elliptocytosis

A

RBCs are ellipse in shape and autosomal dominant

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