Gaucher Disease with Mesenteric Lymphadenopathy: A Case with 13-year Follow-up
نویسندگان
چکیده
Mesenteric lymphadenopathy is a rare manifestation of Gaucher disease (GD) with only 26 cases reported worldwide and its outcome remains largely unknown. In this manuscript, we described a 17-year-old girl with GD who has been treated with standard enzyme replacement therapy (ERT) for 16 years. The follow-up of her mesenteric lymphadenopathy began 13 years ago, which is one of the longest follow-up for this condition worldwide.
منابع مشابه
Ultrastructural features of gaucher disease treated with enzyme replacement therapy presenting as mesenteric mass lesions.
The classical ultrastructural features of Gaucher disease include large numbers of intracytoplasmic, membrane-bound lysosomal inclusions containing characteristic tubular structures on an electron-lucent background, representing the periodic acid schiff (PAS)-positive Gaucher cells identifiable on light microscopy. Following enzyme replacement therapy (ERT), many of the manifestations of the co...
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