原发性浆细胞白血病与淋巴瘤伴浆细胞增多的诊断与鉴别诊断  

Diagnostic and differential diagnostic of primary plasma cell leukemia and lymphoma with increased plasma cell

作　　者：张会超[1] 黄晨[1] 王芃堉 李宏[1] 陈砚凝[1] 张红[1] 丁雅雯[1] 高社军[1] 

机构地区：[1]河北医科大学第四医院血液病实验室,石家庄050011

出　　处：《临床与实验病理学杂志》2017年第5期505-510,共6页Chinese Journal of Clinical and Experimental Pathology

基　　金：河北省科技厅大健康服务和生物医药专项课题(162777243)

摘　　要：目的探讨原发性浆细胞白血病(primary plasma cell leukemia,PPCL)及淋巴瘤伴浆细胞增多的临床病理特征、诊断及鉴别诊断。方法采用临床资料及细胞形态学、流式细胞术、免疫固定电泳及免疫组化Eli Vision两步法等检测7例PPCL及3例淋巴瘤伴浆细胞增多,并进行分析。结果 7例PPCL及3例淋巴瘤伴浆细胞增多的临床特点均以进行性贫血、血小板减少、发热、肝脾及淋巴结肿大最为常见;外周血细胞形态学分类浆细胞比例均大于20%,且伴形态学异常;外周血流式细胞免疫表型显示7例PPCL均表达CD38及CD138,2例表达CD56,2例表达CD20,轻链(Lamda、Kappa)均呈单克隆限制性表达,符合PPCL诊断;3例淋巴瘤伴浆细胞增多CD19、CD45呈弱阳性,CD38、CD138呈阳性,轻链Ig L未见限制性表达,属于正常浆细胞的免疫表型。3例轻链(Ig)未见限制性表达,经淋巴结切除活检病理学检查确诊血管免疫母细胞性T细胞淋巴瘤2例,CD30阳性窦内大B细胞淋巴瘤1例。结论 PPCL与淋巴瘤伴浆细胞增多有相同的临床表现及相似的细胞形态学特征,PPCL的诊断需结合免疫固定电泳及流式细胞免疫表型;而淋巴瘤伴浆细胞增多还需结合淋巴结组织学检查才能确诊。Purpose To investigate the diagnosis, differential diagnosis and clinical manifestation of primary plasma cell leukemia （PPCL） and lymphoma with increased plasma cell. Methods Through clinical data and cell morphology, flow cytometry （FCM） , immunofixation eleetrophoresis and immunohistochemistry of EliVision two-step examination were used to analyze 7 cases of PPCL and 3 cases of lymphoma with increased plasma cell. Results All patients with PPCL and lymphoma with increased plasma cell presented with anemia, thromboeytopenia, fever, liver and spleen and lymph node swelling. The proportion of plasma cells in peripheral blood morphology were larger than 20% , accompanied by morphological abnormality. FCM of peripheral blood showed all 7 cases of PPCL expressed CD38 and CD138, CD56 expression in the 2 cases and CD20 in the 2 cases. The light chain （ Lamda, Kappa） showed a monoclonal restricted expression, which was consistent with the diagnosis of PPCL. CD19 and CD45 were weakly positive in 3 cases of lymphoma with increased plasma cell, CD38 and CD138 were positive, and no restricted expression was found in light chain IgL, wich belonging to the immunophenotypes of normal plasma cells. Of 3 cases of light chain （Ig） without restrictive expression, 2 of them were angioimmunoblastic T-cell lymphoma （ATCL） and 1 case was CD30-positive sinusoidal large B-cell lymphoma （CD30^＋ SLBCL） that confirmed by lymph node biopsy and pathological examination. Conclusion The PPCL and lymphoma with increased plasma cell have the same clinical manifestations and similar morphological characteristics of blood cells. The diagnosis of PPCL should be combined with immunoelectrophoresis and FCM, and the diagnosis of lymphoma with increased plasma cell needs to be confirmed by histological examination of lymph nodes.

关 键 词：原发性浆细胞白血病 淋巴瘤 反应性浆细胞增多症 免疫组织化学 

分 类 号：R446[医药卫生—诊断学] R392.31[医药卫生—临床医学]