Partial splenectomy for massive splenomegaly secondary to Gaucher's disease.
نویسندگان
چکیده
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.
منابع مشابه
Giant cavernous haemangioma of the spleen presenting as massive splenomegaly and treated by partial splenectomy.
Cavernous haemangioma is a rare disorder of the spleen with fewer than 100 cases reported. Only rarely do they attain large sizes. A 36-year-old woman presented with a six-month history of pain in the left hypochondrium and a massive splenomegaly. Ultrasonography, Doppler studies, and computed tomography could not distinguish between a haemangioma and a secondary deposit. Magnetic resonance ima...
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متن کاملSplenomegaly, hypersplenism, and hereditary disorders with splenomegaly
Splenomegaly, sometimes of massive extent, occurs in a large number of hereditary diseases, some relatively prevalent and others, rare to ultra-rare. Because physicians are often unfamiliar with the less common disorders, patients may suffer because of diagnostic delay or diagnostic error and may undergo invasive, non-innocuous procedures such as splenectomy that are potentially avoidable were ...
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عنوان ژورنال:
- Postgraduate medical journal
دوره 67 794 شماره
صفحات -
تاریخ انتشار 1991