| Literature DB >> 25332606 |
Jayastu Senapati1, Anup J Devasia1, Sachin David1, Marie Therese Manipadam2, Sheila Nair2, Giridhara R Jayandharan1, Biju George1.
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.Entities:
Keywords: Diffuse large B cell lymphoma; Primary immunodeficiency syndrome; Wiskott-Aldrich syndrome
Year: 2014 PMID: 25332606 PMCID: PMC4192205 DOI: 10.1007/s12288-014-0377-1
Source DB: PubMed Journal: Indian J Hematol Blood Transfus ISSN: 0971-4502 Impact factor: 0.900