华氏巨球蛋白血症的骨髓病理特点与鉴别

Bone marrow pathologic features and differentiation of Waldenstr（o）m macroglobulinemia

目的 探讨华氏巨球蛋白血症（WM）的骨髓病理特点、诊断与鉴别.方法 19例WM患者行骨髓穿刺（BMA）及骨髓活组织检查（BMB）进行形态学观察,用流式细胞术（FCM）及免疫组织化学（IHC）方法 进行免疫表犁分析.结果 11例BMA示浆细胞样淋巴细胞增生.BMB示19例均见瘤细胞侵犯,其中17例主要为小淋巴细胞增生,2例主要为浆细胞样淋巴细胞增生.4例未见典型的浆细胞样淋巴细胞.骨髓侵犯呈弥漫型12例,结节型4例,间质型3例.FCM示14例瘤细胞CD19^＋、CD20^＋、CD22^＋、CD5^-、CD10^-.IHC示小淋巴细胞及浆细胞样淋巴细胞CD20^＋、Pax5^＋,浆细胞CD38^＋、CD138^＋、CD20^-、Pax5^-.结论 小淋巴细胞增生伴有浆细胞样分化是WM的典型骨髓病理组织学改变,IHC有利于识别淋巴细胞及浆细胞两种不同的细胞成分.形态学与FCM、IHC相结合有助于WM的诊断与鉴别.

Objective To explore the bone marrow pathology ,diagnosis and differential diagnosis of Waldenstrom macroglobulinemia（WM）. Methods 19 WM patients was examined by bone marrow aspiration （BMA） and bone marrow biopsy （BMB） for morphology. Flow cytometry （FCM） and immunohistochemistry （IHC） for immunophenotyping. Results Plasmacytoid lymphocytes were identified in 11 BMA. All of 19 BMB were involved by lymphoma cells. 17 cases showed a predominance of small lymphocytes and 2 of plasmacytoid lymphocytes. Typically plasmacytoid lymphocytes were not seen in 4 cases. Patterns of bone marrow involvement were as follow： diffuse （12 cases）, nodular （4 cases）, interstitial （3 cases）. Immunophenotypically, FCM showed all cases were CD19^＋、CD20^＋、CD22^＋、CD5^-、CD10^-. IHC revealed small lymphocytes and plasmacytoid lymphocytes were Pax5^＋ CD20^＋ and plasma cells were CD38 CD138^＋ CD20^- Pax5^-. Conclusion Small lymphocytes proliferation with plasmacytic differentiation is the typical bone marrow pathologic features of WM. IHC is benefit for identifying lymphocytes and plasma cells components. The Combination of morphology, FCM and IHC is contributive to the diagnosis and differentiation of WM.