华西医学

华西医学

华氏巨球蛋白血症/淋巴浆细胞淋巴瘤 NCCN 新版指南(2018.V1)解读

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华氏巨球蛋白血症(Waldenström’s macroglobulinemia,WM)/淋巴浆细胞淋巴瘤(lymphoplasmacytic lymphoma,LPL)是一种发病率较低的成熟 B 细胞淋巴瘤,WM 以淋巴浆细胞浸润骨髓同时伴血清单克隆性免疫球蛋白 M 增高为特点。近年来随着分子机制的挖掘、检测手段的更新以及新药的研制,WM/LPL 的诊断与治疗取得了较大的进展。为提高临床工作者对该疾病的认识水平、规范其诊断及治疗,美国国立综合癌症网络于 2018 年 3 月更新了 WM/LPL 的诊治指南。该文将对指南重点内容进行解读以使国内读者更好地了解有关内容。

Waldenström’s macroglobulinemia (WM)/lymphoplasmacytic lymphoma (LPL) is a rare mature B-cell neoplasm. WM is characterized by the presence of a lymphoplasmacytic infiltrate in the bone marrow and high serum levels of monoclonal immunoglobulin M protein. With a deeper understanding of molecular mechanisms of the disease, the update of diagnostic approaches and the introduction of novel therapies, the management of WM/LPL has rapidly evolved over the past few years. On March 7th, 2018, National Comprehensive Cancer Network (NCCN) updated the guideline for WM/LPL. This study mainly interpreted the corresponding diagnosis and treatment of WM/LPL in NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®): Waldenström’s Macroglobulinemia/Lymphoplasmacytic Lymphoma (Version 1.2018).

关键词: 华氏巨球蛋白血症; 淋巴浆细胞淋巴瘤; 诊断; 治疗

Key words: Waldenström’s macroglobulinemia; Lymphoplasmacytic lymphoma; Diagnosis; Therapy

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