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

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

Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion

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

Affiliations

  1. Department of Clinical Haematology, Christian Medical College and Hospital, Vellore, 632004 India.
  2. Department of General Pathology, Christian Medical College and Hospital, Vellore, 632004 India.

PMID: 25332606 PMCID: PMC4192205 DOI: 10.1007/s12288-014-0377-1

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.

Keywords: Diffuse large B cell lymphoma; Primary immunodeficiency syndrome; Wiskott-Aldrich syndrome

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