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Case Reports
. 1991 Dec;67(794):1072-4.
doi: 10.1136/pgmj.67.794.1072.

Partial splenectomy for massive splenomegaly secondary to Gaucher's disease

W E Thomas  1 , D A Winfield
Affiliations
Case Reports

Partial splenectomy for massive splenomegaly secondary to Gaucher's disease

W E Thomas et al. Postgrad Med J. 1991 Dec.

Abstract

A 16 year old girl with Type 1 Gaucher's disease presented with massive splenomegaly, hypersplenism and abdominal discomfort. Traditionally hypersplenism has been treated with splenectomy, but this results in a high incidence of overwhelming sepsis and accelerated sphingolipid deposition in both liver and bone. A 90% partial splenectomy was therefore performed leaving a fully vascularized inferior segment of the spleen and resecting 5.8 kg of splenic tissue. The patient made an uneventful recovery with a marked improvement in her haematological parameters and general condition.

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