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Massive Splenomegaly in Gaucher's Disease

Michael F. Appel, MD; Alfred M. Markowitz, MD
JAMA. 1971;217(3):343-344. doi:10.1001/jama.1971.03190030067028.
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To the Editor.—  Gaucher's disease is one of many entities which can be accompanied by a markedly enlarged spleen. The consequences of massive splenomegaly are well known. Depletion of one or more of the cellular elements of the blood is a common indication for splenectomy. In extreme cases, cosmetic disfigurement may occur.The case presented involved both of these problems. We also believe the spleen to represent the largest to be successfully removed.

Report of a Case.—  In a 22-year-old woman, the diagnosis of Gaucher's disease had been made at age 7 1/2 years from bone marrow examination. She was lost to follow-up until age 21 years, when she received several blood transfusions for anemia at another hospital. Splenectomy was advised and she was referred here for consideration of this operation.This asthenic, pale, white woman was in no distress. The abdomen was markedly protuberant and filled in all four

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