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Diffuse large B cell lymphoma in wiskott-Aldrich syndrome: a case report and review of literature.

作者信息

Senapati Jayastu, Devasia Anup J, David Sachin, Manipadam Marie Therese, Nair Sheila, Jayandharan Giridhara R, George Biju

机构信息

Department of Clinical Haematology, Christian Medical College and Hospital, Vellore, 632004 India.

Department of General Pathology, Christian Medical College and Hospital, Vellore, 632004 India.

出版信息

Indian J Hematol Blood Transfus. 2014 Sep;30(Suppl 1):309-13. doi: 10.1007/s12288-014-0377-1. Epub 2014 Apr 11.

Abstract

Wiskott-Aldrich syndrome (WAS) is an X linked rare primary immunodeficiency syndrome with an increased propensity for infection, autoimmunity and malignancy. Here we report a male child, who was diagnosed with WAS at 1 year of age following evaluation for symptomatic thrombocytopenia and eczematous skin lesions. He presented later with lymphadenopathy, which was consistent with diffuse large B cell lymphoma on histopathology. He received 6 cycles of R-CHOP chemotherapy for the same and is presently in remission after 6 months. We review the major publications of lymphoma in WAS and discuss the pathological findings, treatment and prognosis of lymphoma in WAS.

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