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Waldenstrom巨球蛋白血症临床诊断特点

Clinical diagnosis features of patients with Waidenstrom macroglobulinemia
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摘要 目的探讨Waldenstrom巨球蛋白血症(WM)的临床特点,提高对这种痰病的认识及诊断水平。方法对我院2001年1月至2010年4月确诊为WM的15例患者临床资料进行回顾性分析。结果WM好发于老年男性,中住发病年龄62岁(53—79),男/女=4/1。患者多以疲乏、贫血和出血倾向就诊,主要表现为头晕、乏力,消瘦,脾大,淋巴结肿大和高粘综合征。血清单克隆1gM〉30g/L15例(75%),血清蛋白固定电泳均可见IgM轻链条带。20例骨髓活检和11例淋巴结活检均经病理组织形态和免疫组织化学诊断为LPL,骨髓及淋巴结瘤细胞由小淋巴细胞、浆细胞样淋巴细胞及浆细胞组成。侵犯骨筵的方式多为弥漫型(55%,11/20),间质型(25%,5/20),结节型(20%,4/20)。FCM示14例瘤细胞CD19+、CD20+、cD22+、CD3-、CD5-、cD10-。免疫组化示小淋巴细胞及浆细胞样淋巴细胞cD20+、CD79a+、Pax5+,浆细胞cD38+、CD138+、Pax5-,3例CD5弱表达,所有瘤细胞CD23-、CD10-。结论Waldenstrom巨球蛋白血症的本质为LPL。小淋巴细胞增牛伴有浆细胞样分化是WM的典型骨髓病理组织学改变,免疫组化染色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、免疫组化染色相结合有助于WM的诊断与鉴别。 Objective To explore the clinical characteristics of Waldenstrom macroglobulinemia and improve the level of diagnosis. Method Analyze retrospectively the 20 patients with WM in our hospital during in January 2001 to April 2010. Result WM is familiar in old and male patiens and the median age was 62(53 - 79), M/F = 4/1. Primary -clinical representations were fatigue, lose weight, splenomegaly, lymphadenopathy, and hyper viscosity syndrome. Assistant examination showed that 15 cases (75 % ) serum monoclonal 1gM 〉 30g/L. Basing on the histology and immunohistochemistry findings, the diagnosis Was made in 20 bone marrow and 11 lymph node biopsies respectively. Histologically, the bone marrow and lymph node specimens composed of small lymphoeytes, plasmacytoid lymphocytes and plasma cells. The most frequent pattern of bone marrow involvement Was diffuse in appearance( 55%, 11/20) ,while nodular andinterstitial patterns were less common (25%, 5/20 and 20%, 4/20,respectively). FCM showed all eases were CD19 + , CD20 + , cD22 + , CD3 - ,CD5 - , cD10 - ; IHC revealed small lymphocytes and plasmacytoid lymphoeytes were cD20 + , GD79a + , Pax5 + and plasma cells were cD38 + ,CD138 + , Pax5 - , three cases weakly expressed CDS. All of the proliferative cells expressed CD23 - , CD10- . Conclusion Waldenstrom macroglobulinemia is LPL. Small lymphocytes proliferation with plasmaeytie differentiation is the typical bone marrowpathologic 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.
作者 潘露
出处 《医学检验与临床》 2012年第2期56-59,共4页 Medical Laboratory Science and Clinics
关键词 WALDENSTROM巨球蛋白血症 临床诊断 免疫分型 淋巴瘤 Waldenstrom macroglobulinemia Clinical diagnosis Immunophenotyping Lymphoma
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