Lymphomatoid granulomatosis with splenomegaly and pancytopenia

Lymphomatoid granulomatosis (LG) is an angiocentric lymphoproliferative disease. It usually involves lung, skin, and central nervous system, but splenomegaly and pancytopenia are the rare manifestations of the disease. We report a 15-year-old boy presented with fever, dry cough and dyspnea from two...

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Bibliographic Details
Published in:Zhongguo fei ai za zhi 2010, Vol.13 (1), p.84-86
Main Authors: Halvani, Abolhasan, Owlia, Mohammad Bagher, Sami, Ramin
Format: Article
Language:English
Subjects:
Adolescent
Case Report
Humans
Lymphomatoid Granulomatosis - diagnosis
Lymphomatoid Granulomatosis - diagnostic imaging
Lymphomatoid Granulomatosis - drug therapy
Male
Pancytopenia - diagnosis
Pancytopenia - diagnostic imaging
Pancytopenia - drug therapy
Radiography
Splenomegaly - diagnosis
Splenomegaly - diagnostic imaging
Splenomegaly - drug therapy
病例报道
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Summary:Lymphomatoid granulomatosis (LG) is an angiocentric lymphoproliferative disease. It usually involves lung, skin, and central nervous system, but splenomegaly and pancytopenia are the rare manifestations of the disease. We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly. Then he manifested neurologic signs such as seizure, aphasia and right-sided hemiplegia. Chest X-ray and CT scan revealed bilateral pulmonary nodules predominantly in lower lobes and peripheral lung fields. Laboratory exams showed pancytopenia. Skin biopsy was done, and histopathological examination and immunohistochemistry evaluation confirmed lymphomatoid granulomatosis. He was treated with steroid and cyclophosphamide but succumbed by neurologic involvement.
ISSN:1009-3419
1999-6187
DOI:10.3779/j.issn.1009-3419.2010.01.17