Senapati Jayastu, Devasia Anup J, David Sachin, Manipadam Marie Therese, Nair Sheila, Jayandharan Giridhara R, George Biju
Department of Clinical Haematology, Christian Medical College and Hospital, Vellore, 632004 India.
Department of General Pathology, Christian Medical College and Hospital, Vellore, 632004 India.
Indian J Hematol Blood Transfus. 2014 Sep;30(Suppl 1):309-13. doi: 10.1007/s12288-014-0377-1. Epub 2014 Apr 11.
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.