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

J Senapati, AJ Devasia, S David… - Indian Journal of …, 2014 - Springer
Indian Journal of Hematology and Blood Transfusion, 2014Springer
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 …
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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