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目的:探讨淋巴结淋巴浆细胞淋巴瘤/华氏巨球蛋白血症（n-LPL/WM）的临床病理学特点及预后。方法:收集郑州大学第一附属医院2009年5月至2020年1月诊断的n-LPL/WM 19例，分析其临床、形态学、免疫组织化学及免疫球蛋白（Ig）基因重排情况（BIOMED-2法），用Sanger测序法检测MYD88 L265P基因突变情况，并随访患者。结果:患者男性15例，女性4例，中位年龄61岁（年龄范围43~82岁）；华氏巨球蛋白血症（WM）14例，淋巴浆细胞淋巴瘤（LPL） 5例；临床表现最多见乏力、疲倦（9/19）及B症状（11/19），多数（16/18）患者全身多发淋巴结肿大；18例临床均处于进展期；血清M蛋白：IgM型15例、IgG及IgA型各1例、无分泌型2例；17例（17/18）骨髓受累及。形态学分为典型组（9例）及不典型组（10例），典型组淋巴结结构保留，以浆样淋巴细胞增生为主或小淋巴细胞、浆样淋巴细胞及浆细胞混合增生，无滤泡树突细胞（FDC）网增生及滤泡植入；不典型组可见淋巴结结构破坏（5例）、小淋巴细胞增生为主（6例）、FDC网增生和（或）滤泡植入（6例）、边缘带细胞分化（4例）及弥漫性淀粉样变性（1例），两组均可见含铁血黄素沉积（19例）、结外脂肪组织浸润（19例）及间质条带状硬化（9例）。免疫组织化学：肿瘤性B细胞均表达CD20、CD79α，肿瘤性浆细胞均表达CD38、CD138及MUM1，8例（8/8）轻链限制，7例中5例表达IgM、2例分别表达IgG及IgA，4例CD23弱阳性，Ki-67阳性指数10%~30%；两组临床病理学特征及预后差异均无统计学意义（n P>0.05）；18例（18/18）MYD88 L265P突变。中位随访时间61个月，11例存活，8例死亡，5年存活率21.1%。n 结论:n-LPL/WM少见，临床分期常较高，形态学易与其他小B细胞淋巴瘤伴浆样分化混淆，结合临床表现、血清M蛋白值、免疫组织化学、骨髓活检、流式细胞学及MYD88 L265P突变检测等综合分析可明确诊断。n-LPL/WM患者可能预后不良，有待扩大样本进一步研究。“,”Objective:To study the clinicopathological features and prognosis of nodal lymphoplasmacytic lymphoma/Waldenstrom′s macroglobulinemia (n-LPL/WM).Methods:A total of 19 cases of n-LPL/WM were collected from May 2009 to January 2020 at First Affiliated Hospital of Zhengzhou University. The clinicopathologic features, immunophenotype, Ig gene rearrangement (BIOMED-2), MYD88 L265P mutation status (by Sanger sequencing) and follow-up data (by telephone) were analyzed.Results:There were 15 males and 4 females with a median age of 61 years (range 43 to 82 years). There were 14 WM and five LPL. The most common symptoms were weakness, fatigue (9/19) and B symptoms (11/19). Majority of the patients (16/18) presented with systemic multiple lymphadenopathies. Eighteen patients presented at advanced stages (Ⅲ/Ⅳ stage). Serum M protein status was IgM (15 cases), IgG (1 case), IgA (1 case) and no-secretory type (2 cases). Seventeen patients had bone marrow involvement. Morphologically, all 19 cases were divided into two groups: typical group (9 cases) or atypical group (10 cases). In the typical group, the structures of the lymph nodes were preserved; the neoplastic cells were predominantly plasmacytoid lymphocytes or mixed small lymphocytes, plasmacytoid lymphocytes and plasma cells, without proliferation of FDC network and follicular implantation. In the atypical group, the tumor showed effaced nodal architecture (5 cases), mainly proliferation of small lymphocytes (6 cases), FDC proliferation and/or follicular implantation (6 cases), marginal zone B cell differentiation (4 cases) and diffuse amyloidosis (1 case). Hemosiderin deposition (19 cases), infiltration of fatty tissue (19 cases) and interstitial sclerosis (9 cases) were commonly seen in both groups. Immunohistochemically, the neoplastic B cells expressed CD20 and CD79α, and the neoplastic plasma cells were positive for CD38, CD138 and MUM-1; eight cases showed light chain restriction; of the seven detected cases, five expressed IgM and the other two expressed IgG and IgA respectively; four cases expressed CD23 weakly, Ki-67 index was 10%-30%. MYD88 L265P mutation was seen in 18/18 cases. There was no significant difference in clinicopathologic features and prognosis between the two groups (n P>0.05). The median follow-up time was 61 months, 11 patients were alive, while eight died; the 5-year survival rate was 21.1%.n Conclusions:n-LPL/WM is rare, but patients usually present in advanced stages. It is easily confused with other small B-cell lymphomas with plasma cell differentiation, especially basing on morphologic features alone; thus the accurate diagnosis of n-LPL/WM requires a combination of clinical features, serum M protein, immunohistochemistry, bone marrow morphology,flow cytometry and MYD88 L265P mutation status etc. The prognosis of n-LPL/WM may be not very good, and further studies with more cases are needed.