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浆母细胞淋巴瘤是一种临床罕见、侵袭性极强的B细胞淋巴瘤,多发生在HIV感染的患者,也可见于HIV阴性的免疫功能正常或受抑的人群,常与EB病毒感染相关。由于其特殊的形态学及免疫表型特征、化疗耐药及复发率高的临床特点,诊断及治疗仍极具挑战性,总体预后极差。本文简要总结了PBL在流行病学、病理学特征、临床表现、诊断、治疗及预后因素等方面的进展,期望为PBL的临床诊治提供帮助。
Plasmacytoid lymphoma is a clinically rare and highly aggressive B cell lymphoma that occurs in HIV-infected patients as well as in HIV-negative individuals with normal or suppressed immune function and is often associated with Epstein-Barr virus infection . Because of its special morphological and immunophenotypic characteristics, chemoresistance and high recurrence rate of clinical features, diagnosis and treatment is still very challenging, the overall prognosis is very poor. This article briefly summarizes the PBL in the epidemiology, pathological features, clinical manifestations, diagnosis, treatment and prognostic factors, etc., and hope to help the clinical diagnosis and treatment of PBL.