Chen Yi-Hua, Tallman Martin S, Goolsby Charles, Peterson LoAnn
Department of Pathology, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA.
Am J Clin Pathol. 2006 Feb;125(2):251-9. doi: 10.1309/PMQX-VY61-9Q8Y-43AR.
Hairy cell leukemia (HCL) exhibits a characteristic immunophenotypic profile that is strongly positive for pan-B-cell markers; positive for CD103, CD11c. and CD25; and usually negative for CD5, CD10, and CD23. We evaluated 35 HCL cases and identified atypical immunophenotypes in 12 cases (34%), including CD103- in 2 (6%), CD25- in 1 (3%), CD10+ in 5 (14%), and CD23+ in 6 (17%) cases. Among these cases one was CD103-/CD10+ and one was CD10+/CD23+. All available specimens from the 12 cases were reviewed and showed morphologic features characteristic for HCL. The initial clinical information was reviewed and showed no significant differences with that reported for typical HCL. Of the 12 cases, 11 patients received purine analogue therapy and achieved complete remissions. Our study indicates that it is not uncommon for HCL to display an unusual immunophenotype, including negativity for CD103 or CD25. Recognizing the variability of immunophenotype and correlating with morphologic and clinical features are essential for establishing an accurate diagnosis of HCL.
毛细胞白血病(HCL)呈现出特征性的免疫表型特征,对泛B细胞标志物呈强阳性;对CD103、CD11c和CD25呈阳性;通常对CD5、CD10和CD23呈阴性。我们评估了35例HCL病例,在12例(34%)中鉴定出非典型免疫表型,包括2例(6%)CD103阴性、1例(3%)CD25阴性、5例(14%)CD10阳性和6例(17%)CD23阳性。在这些病例中,1例为CD103阴性/CD10阳性,1例为CD10阳性/CD23阳性。对这12例病例的所有可用标本进行复查,均显示出HCL的形态学特征。复查初始临床信息,与典型HCL报告的情况无显著差异。这12例病例中,11例患者接受了嘌呤类似物治疗并实现完全缓解。我们的研究表明,HCL出现异常免疫表型并不罕见,包括CD103或CD25阴性。认识到免疫表型的变异性并与形态学和临床特征相关联对于准确诊断HCL至关重要。