Robbins B A, Ellison D J, Spinosa J C, Carey C A, Lukes R J, Poppema S, Saven A, Piro L D
Department of Pathology, Scripps Clinic and Research Foundation, La Jolla, CA 92037.
Blood. 1993 Aug 15;82(4):1277-87.
Recent immunophenotypic studies of hairy cell leukemia (HCL) have suggested specific patterns of immunoreactivity that may aid in diagnosis. We studied peripheral blood (PB) from 161 cases of HCL using two-color direct immunofluorescence flow cytometry and an extended panel of antibody combinations. Circulating hairy cells were identified by immunophenotypic features in 92% of the cases and could be detected even when representing < or = 1% of circulating lymphocytes. The 133 cases with > or = 2% detectable hairy cells were analyzed in detail. HCL showed a uniform and unique B-cell phenotype, with each of the following features identified in 99% to 100% of cases: (1) positive staining for B-ly7, coexpressed with CD19; (2) very intense, uniform expression of CD11c, with CD19; (3) moderately intense staining for CD25, with CD19; (4) very intense staining for CD22; (5) moderate to very intense staining for CD20; and (6) moderately intense monoclonal surface Ig. Phenotypic variability existed in expression of CD10 (26%) and CD5 (4%). Based on these features, HCL was easily distinguished from 50 cases of chronic lymphocytic leukemia (CLL). Although CLL exhibited frequent expression of CD11c (74%) and CD25 (68%), the intensity of staining was significantly less than HCL. Furthermore, CLL was uniformly positive for CD5 and showed weak staining for CD20, CD22, and surface Ig. B-ly7 proved to be the most specific marker, reacting with 100% of HCL cases, but absent in all cases of CLL. We conclude that two-color flow cytometry with specific antibody combinations is an efficacious method for characterization and sensitive detection of hairy cells in PB. Application of the phenotypic criteria described should help to increase accuracy in diagnosis of HCL.
近期对毛细胞白血病（HCL）的免疫表型研究表明，特定的免疫反应模式可能有助于诊断。我们使用双色直接免疫荧光流式细胞术和一组扩展的抗体组合，对161例HCL患者的外周血（PB）进行了研究。通过免疫表型特征在92%的病例中识别出循环毛细胞，即使其占循环淋巴细胞的比例≤1%也能被检测到。对133例可检测到≥2%毛细胞的病例进行了详细分析。HCL呈现出一致且独特的B细胞表型，在99%至100%的病例中均具有以下特征：（1）B-ly7染色阳性，与CD19共表达；（2）CD11c与CD19共表达，表达非常强烈且均匀；（3）CD25与CD19共表达，染色中等强度；（4）CD22染色非常强烈；（5）CD20染色中等至非常强烈；（6）单克隆表面Ig染色中等强度。CD10（26%）和CD5（4%）的表达存在表型变异性。基于这些特征，HCL很容易与50例慢性淋巴细胞白血病（CLL）区分开来。虽然CLL经常表达CD11c（74%）和CD25（68%），但其染色强度明显低于HCL。此外，CLL的CD5均为阳性，而CD20、CD22和表面Ig染色较弱。B-ly7被证明是最具特异性的标志物，与100%的HCL病例反应，但在所有CLL病例中均不存在。我们得出结论，使用特定抗体组合的双色流式细胞术是对外周血中毛细胞进行特征描述和灵敏检测的有效方法。应用所描述的表型标准应有助于提高HCL诊断的准确性。
