Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society · 2010
Hodgkin's and non-Hodgkin's lymphomas occurring in two brothers with Wiskott-Aldrich syndrome and review of the literature.
Клинический случайСтатьяОбзорНазвание и аннотация: Перевод пока не подготовлен
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Du Shouying, Scuderi Richard, Malicki Denise M, Willert Jennifer, Bastian John, Weidner Noel
Аннотация
Approximately 13% of patients with Wiskott-Aldrich syndrome (WAS), a primary immune deficiency, develop malignant tumors, the predominant form being non-Hodgkin's lymphoma. Previously, only 4 cases of Hodgkin's lymphoma have been reported in WAS patients. Herein, we review the literature of WAS-related lymphomas and report 2 brothers with WAS who both developed lymphomas; one developed Epstein-Barr virus (EBV)-driven diffuse large B-cell lymphoma, and one developed EBV-negative classical Hodgkin's lymphoma. In contrast to many of the previously reported lymphomas in WAS patients, these lymphomas were extensively evaluated by means of molecular, flow cytometric, and immunohistochemical methods. Both brothers died shortly after diagnosis, despite aggressive therapy. The occurrence of 2 distinct forms of lymphomas in these brothers underscores the interplay between genetic susceptibility and environmental exposure in lymphoma pathogenesis.