Cornfield D B, Mitchell Nelson D M, Rimsza L M, Moller-Patti D, Braylan R C
Department of Pathology and Laboratory Medicine, University of Florida College of Medicine, Gainesville, Florida 32610, USA.
Am J Hematol. 2001 Aug;67(4):223-6. doi: 10.1002/ajh.1120.
The diagnosis of hairy cell leukemia (HCL) has traditionally been based on microscopic means. Immunophenotypic analysis of peripheral blood by flow cytometry is not widely recognized as a method for diagnosing HCL, perhaps due to the expectation of low yield of neoplastic cells in patients who are characteristically leukopenic. The abnormal coexpression of CD103, CD25, and intense CD11c and CD20 on monotypic, slightly large B-lymphocytes has previously been shown to be highly characteristic of HCL. We wished to determine if this pattern was valuable in the diagnosis of HCL in leukopenic patients with low levels of neoplastic cells in the peripheral blood. The abnormal immunophenotype above was observed in 25 peripheral blood specimens from patients with unexplained cytopenias or suspected lymphoproliferative processes. Ten of the 25 blood samples exhibited this abnormal phenotype in less than 5% of circulating leukocytes (ranging from <1% to 4%). All 10 patients had other manifestations of HCL, including cytopenias (mean white blood cell count, 1.8 x 10(3)/mm(3); hemoglobin, 11.0 gm/dl; platelets, 74 x 10(3)/mm(3)), splenomegaly, and typical bone marrow morphologic changes. Eight of the 10 patients achieved an excellent response to one course of 2-CDA, with significant improvement of cytopenias (mean white blood cell count: 5.3 x 10(3)/mm(3); hemoglobin: 14.4 g/dl; platelets: 181 x 10(3)/mm(3)) and regression of splenomegaly. One patient had a partial response to alpha interferon and a subsequent complete response to 2-CDA, and one died during treatment. In conclusion, flow cytometric immunophenotyping of peripheral blood is capable of detecting low levels of circulating malignant cells in HCL, even in leukopenic patients. As such, it can be a very useful, non-invasive tool in the diagnosis of this disorder.
毛细胞白血病（HCL）的诊断传统上基于显微镜检查方法。通过流式细胞术对外周血进行免疫表型分析尚未被广泛认可为诊断HCL的方法，这可能是因为预期在典型白细胞减少的患者中肿瘤细胞的检出率较低。先前已表明，在单型、略大的B淋巴细胞上CD103、CD25的异常共表达以及强烈的CD11c和CD20是HCL的高度特征性表现。我们希望确定这种模式在诊断外周血中肿瘤细胞水平较低的白细胞减少患者的HCL时是否有价值。在25例来自不明原因血细胞减少或疑似淋巴增殖性疾病患者的外周血标本中观察到上述异常免疫表型。25份血样中有10份在循环白细胞中不到5%的细胞中表现出这种异常表型（范围从<1%到4%）。所有10例患者均有HCL的其他表现，包括血细胞减少（平均白细胞计数为1.8×10³/mm³；血红蛋白为11.0 g/dl；血小板为74×10³/mm³）、脾肿大和典型的骨髓形态学改变。10例患者中有8例对一个疗程的2 - CDA治疗反应良好，血细胞减少有显著改善（平均白细胞计数：5.3×10³/mm³；血红蛋白：14.4 g/dl；血小板：181×10³/mm³）且脾肿大消退。1例患者对α干扰素部分反应，随后对2 - CDA完全反应，1例在治疗期间死亡。总之，外周血流式细胞术免疫表型分析能够检测出HCL中循环恶性细胞的低水平，即使在白细胞减少的患者中也是如此。因此，它可以成为诊断这种疾病的非常有用的非侵入性工具。
