淋巴浆细胞性淋巴瘤伴Waldenstrom巨球蛋白血症的临床病理和免疫表型分析

摘要

Objective To investigate the clinicopathologic features of lymphoplasmacytic lymphoma (LPL) with Waldenstrom's macroglobulinemia (WM) and to evaluate the usefulness of immunophenotype analysis in diagnosis and differential diagnosis of the tumor. Methods A total of 40 cases of LPL with WM diagnosed according to the 2008 World Health Organization classification of tumors of hematopoietic and lymphoid tissues were analyzed using immunophenotype and follow-up information. Results The mostly common initial clinical presentations were non-specific symptoms, such as fatigue, anemia and hemorrhage. Lymphadenopathy, splenomegaly and hepatomegaly were found in 42.5%, 20.0% and 12.5% of the patients respectively. The pattern of bone marrow involvement included mixed type (47.2%), diffuse type (41.7%) and interstitial type (11.1%). The nodal architecture was completely destroyed in one case and partially effaced with residual germinal centers and dilated sinuses in 8 cases. All of the neoplastic cells expressed CD20 and CD79a. Neoplastic plasma cells were positive for CD138 and CD79a. No cases expressed CD5. Four cases weakly expressed CD23. No significant prognosis related factors were identified in the survival analysis. Conclusions LPL with WM is a rare indolent small B-cell lymphoma, which is commonly seen, in older male patients. The tumor frequently involves bone marrow and shows various clinical manifestations. Combination analyses of the bone marrow biopsy histology, immunophenotypic study and clinical data, especially the serum examination are important for the diagnosis of LPL with WM.%目的 探讨淋巴浆细胞性淋巴瘤伴Waldenstrom巨球蛋白血症的临床病理学特征及预后,了解其免疫表型在病理诊断和鉴别诊断中的作用.方法 根据2008版WHO淋巴造血组织肿瘤分类收集40例淋巴浆细胞性淋巴瘤伴Waldenstrom巨球蛋白血症患者临床及随访资料,对标本进行免疫表型检测(SP法)及PAS、甲苯胺蓝、刚果红特殊染色.结果 患者年龄40～90岁,中位年龄64岁.患者多以疲乏、贫血和出血倾向就诊.淋巴结、脾脏和肝脏肿大比率分别为42.5%、20.0%和12.5%.36例骨髓浸润模式表现为混合型(17例)、弥漫型(15例)和间质型(4例).9例淋巴结,1例结构完全破坏,8例部分破坏,可见扩张的淋巴窦和残存的淋巴滤泡.骨髓组织与淋巴结组织免疫表型结果基本一致.肿瘤性小淋巴细胞表达CD20和CD79a,浆细胞表达CD79a和CD138.所有病例均不表达CD5,仅4例弱表达CD23.生存分析显示不同的骨髓浸润模式、白蛋白值、血小板计数、白细胞计数、血清IgM值对患者生存时间均未见影响.结论 淋巴浆细胞性淋巴瘤伴Waldenstrom巨球蛋白血症为一种少见的好发于老年人的惰性小B细胞淋巴瘤,临床表现多样化.骨髓活检形态学观察、免疫表型分析及临床资料(尤其是血清学检查)对于其临床病理诊断至关重要.