淋巴浆细胞淋巴瘤和华氏巨球蛋白血症：临床病理特征和鉴别诊断。
Lymphoplasmacytic lymphoma and Waldenström macroglobulinaemia: clinicopathological features and differential diagnosis.
机构信息
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
出版信息
Pathology. 2020 Jan;52(1):6-14. doi: 10.1016/j.pathol.2019.09.009. Epub 2019 Nov 22.
Lymphoplasmacytic lymphoma (LPL) represents a distinct type of mature B-cell lymphoma with a substantial subset of cases being associated with Waldenström macroglobulinaemia (WM), defined as primarily bone marrow involvement and the presence of an IgM monoclonal paraprotein. MYD88 L265P mutation, although not specific, is present in the vast majority (>90%) of LPL cases and sheds light on the potential pathogenesis of this disease. This review offers an overview of current knowledge on the pathogenesis, clinical presentations, histological features and immunophenotype of LPL and WM. In addition, the differential diagnosis of LPL and WM from other mature B cell neoplasms is highlighted with a focus on distinction from marginal zone lymphoma and plasma cell neoplasms.
淋巴浆细胞淋巴瘤（LPL）代表一种独特的成熟 B 细胞淋巴瘤，其中相当一部分病例与 Waldenström 巨球蛋白血症（WM）相关，其定义为主要累及骨髓和存在 IgM 单克隆副蛋白。MYD88 L265P 突变虽然不具有特异性，但存在于绝大多数（>90%）的 LPL 病例中，为该疾病的潜在发病机制提供了线索。这篇综述概述了 LPL 和 WM 在发病机制、临床表现、组织学特征和免疫表型方面的最新知识。此外，还强调了从其他成熟 B 细胞肿瘤中鉴别诊断 LPL 和 WM 的重要性，重点是与边缘区淋巴瘤和浆细胞瘤的鉴别。
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