[Polyclonal B-cell lymphocytosis with clinical and hematological features resembling hairy cell leukemia].
[Polyclonal B-cell lymphocytosis with clinical and hematological features resembling hairy cell leukemia].
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[多克隆 B 细胞淋巴细胞增多症，其临床和血液学特征类似于毛细胞白血病]。
DOI：
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发表时间：
1998
期刊：
影响因子：
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通讯作者：
T. Machii
中科院分区：
文献类型：
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作者：
H. Kanbayashi;K. Nagata;T. Tanaka;S. Matsuda;H. Sakuma;Y. Maruyama;T. Machii
1例49岁男性因脾肿大和淋巴细胞增多症而住进本院。他没有重大的过往历史，也不吸烟。体格检查发现巨大的脾肿大，没有可触及的浅表淋巴结。血液学检查显示血红蛋白浓度为10.5g/dl，血小板计数为9.8×10(4)/微升，白细胞计数为21.2×10(3)/微升，淋巴细胞异常率为70%。在May-Giemsa染色的血片中，异常淋巴细胞胞核圆形，胞浆苍白，边缘呈锯齿状。相差显微镜和扫描电子显微镜检查显示有许多长的表面绒毛。这些细胞中耐酒石酸酸性磷酸酶活性为阴性。异常淋巴细胞有CD5-、CD10-、CD11a+、CD11c+、CD19+、CD20+、CD22+表型。这些特征类似于描述的一种变异形式的毛细胞白血病(HCL-日本变异)。然而，对Ig基因重排和Sig表达的研究表明，B细胞呈多克隆增殖。根据这些发现，该病例被诊断为毛状B细胞淋巴增生性疾病，这是一种最近描述的以多克隆性B细胞淋巴细胞症为特征的疾病，其特征类似于HCL-日本变异型。对爱泼斯坦-巴尔病毒抗体的血清学分析表明，过去有过感染。脾切除可缓解贫血和血小板减少，但不能缓解淋巴细胞增多。
A 49-year-old man was admitted to our hospital for investigation of splenomegaly and lymphocytosis. He had no significant past history and was not a smoker. Physical examination revealed massive splenomegaly and no palpable superficial lymph nodes. Hematological examination showed a hemoglobin concentration of 10.5g/dl, a platelet count of 9.8 x 10(4)/microliter, and a leukocyte count of 21.2 x 10(3)/microliter with 70% abnormal lymphocytes. In May-Giemsa stained blood films, the abnormal lymphocytes had round nuclei, abundant, pale cytoplasm, and slightly serrated edges. Phase-contrast microscopic and scanning electron microscopic examinations revealed many long surface villi. Tartrate-resistant acid phosphatase activity in these cells was negative. The abnormal lymphocytes had a CD5-, CD10-, CD11a+, CD11c+, CD19+, CD20+, CD22+ phenotype. These features were similar to those described for a variant form of hairy cell leukemia (HCL-Japanese variant). However, studies of Ig gene rearrangement and expression of sIg revealed a polyclonal proliferation of B cells. On the basis of these findings, this case was diagnosed as hairy B-cell lymphoproliferative disorder, a recently described condition characterized by polyclonal B-cell lymphocytosis and features resembling HCL-Japanese variant. Serological assays for antibodies against Epstein-Barr virus suggested a past infection. Splenectomy alleviated the anemia and thrombocytopenia, but not the lymphocytosis.