Machii T, Yamaguchi M, Inoue R, Tokumine Y, Kuratsune H, Nagai H, Fukuda S, Furuyama K, Yamada O, Yahata Y, Kitani T
Department of Hematology and Oncology, Osaka University Medical School, Japan.
Blood. 1997 Mar 15;89(6):2008-14.
Polyclonal B lymphocytosis was found in four patients having clinical and hematologic features resembling those of hairy cell leukemia (HCL). All four patients were women between 37 and 67 years of age. Three patients had splenomegaly. Lymphadenopthy was absent or slight. Persistent lymphocytosis was seen in all the patients, and anemia and/or thrombopenia was observed in three of the patients. Abnormal lymphocytes have long microvilli and prominent membranous ruffles on their surfaces. Bone marrow aspirates and biopsy specimens showed increased numbers of abnormal lymphocytes with round nuclei and abundant pale cytoplasm. Although these findings were similar to those of HCL, studies of Ig gene rearrangements and expression showed the polyclonal proliferation of B cells. We called this new disease hairy B-cell lymphoproliferative disorder (HBLD). All four patients exhibited a polyclonal increase in serum IgG. The morphology of the cells in HBLD was more similar to that of leukemia cells of a variant form of HCL (HCL-Japanese variant) than to typical HCL cells. The surface IgG+, CD5-, CD11c+, CD22+, CD24-, CD25- phenotype and the weak tartrate-resistant acid phosphatase activity in the cells were identical to those of HCL cells of the Japanese variant. Our findings suggest that the B cells in HBLD are the nonmalignant counterpart of leukemic B cells in HCL-Japanese variant.
在四名具有类似于毛细胞白血病（HCL）临床和血液学特征的患者中发现了多克隆B淋巴细胞增多症。所有四名患者均为37至67岁的女性。三名患者有脾肿大。无或仅有轻微淋巴结病。所有患者均出现持续性淋巴细胞增多，三名患者观察到贫血和/或血小板减少。异常淋巴细胞表面有长微绒毛和明显的膜皱褶。骨髓穿刺和活检标本显示，圆形核和丰富淡染细胞质的异常淋巴细胞数量增加。尽管这些发现与HCL相似，但Ig基因重排和表达研究显示B细胞呈多克隆增殖。我们将这种新疾病称为毛细胞B淋巴细胞增殖性疾病（HBLD）。所有四名患者血清IgG均呈多克隆增加。HBLD中细胞的形态与HCL变异型（HCL-日本变异型）白血病细胞的形态比典型HCL细胞更相似。细胞表面IgG+、CD5-、CD11c+、CD22+、CD24-、CD25-表型以及细胞中弱抗酒石酸酸性磷酸酶活性与日本变异型HCL细胞相同。我们的研究结果表明，HBLD中的B细胞是HCL-日本变异型白血病B细胞的非恶性对应物。
