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Diffuse Large B Cell Lymphoma in Wiskott-Aldrich Syndrome: A Case Report and Review of Literature

机译:Wiskott-Aldrich综合征弥漫性大B细胞淋巴瘤:病例报告和文献复习。

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摘要

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.
机译:Wiskott-Aldrich综合征(WAS)是一种X连锁罕见的原发性免疫缺陷综合征,具有增加的感染,自身免疫和恶性倾向。在这里,我们报告了一名男婴,该男婴经过评估有症状的血小板减少症和湿疹性皮肤病变后,在1岁时被诊断出患有WAS。他随后出现淋巴结肿大,这与组织病理学上弥漫的大B细胞淋巴瘤一致。他同样接受了6个周期的R-CHOP化疗,目前在6个月后缓解。我们回顾了WAS中淋巴瘤的主要出版物,并讨论了WAS中淋巴瘤的病理发现,治疗和预后。

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