背景:毛状细胞白血病(HCL)是一种罕见的成熟B细胞淋巴增生性疾病,主要见于男性,以血细胞减少为特征,脾肿大,骨髓纤维化,以及外周血中显示细胞质毛状突起的非典型淋巴样细胞的存在,骨髓,还有脾脏.免疫表型(IPT)谱显示B细胞的克隆扩增,其中CD19、CD20和CD22显示明亮的表达。诊断需要CD103、CD123、CD25和CD11c中的两个毛细胞标志物为阳性。在大多数情况下,HCL变体(HCL-v)具有不同的IPT谱,其中CD25为阴性。
目的:目的是研究经典HCL和HCL变体的血液学和IPT。
方法:本横断面研究包括2015年1月1日至2022年12月31日在印度北部一家三级保健医院进行的8年回顾性诊断的所有HCL病例。纳入研究的患者是那些免疫分型的患者;也就是说,流式细胞术和/或免疫组织化学(IHC)进行诊断。骨髓载玻片,IHC幻灯片,和流式细胞术IPT进行了综述。
结果:该研究包括13名被诊断患有HCL的患者,其中12个是经典HCL,一个是HCL变体(HCL-v)。在经典的HCL中,通过流式细胞术对10例患者进行IPT,而在两名患者中,这是由IHC完成的。所有经典HCL患者的CD19、CD20和CD22均为阳性(分别为10/10、10/10和5/5),而CD123,CD103,CD25和CD11C在100%呈阳性,89%,80%,100%的案例,分别。一名HCL-v患者CD103和CD123阳性,而CD25和CD123为阴性。
结论:HCL的诊断需要多管齐下的方法。临床特征的使用,形态学,和免疫表型结合辅助技术提供了更高的诊断准确性,并使其与其他B细胞淋巴增殖性疾病(BCLPDs)区分开来,导致更好的患者管理和治疗。
BACKGROUND: Hairy cell leukemia (HCL) is a rare lymphoproliferative disorder of the mature B-cells, mostly seen in men, and is characterized by cytopenia, splenomegaly, myelofibrosis, and the presence of atypical lymphoid cells showing the cytoplasmic hairy projection in the peripheral blood, bone marrow, and spleen. The immunophenotypic (IPT) profile shows the clonal expansion of B-cells with CD19, CD20, and CD22 showing bright expression. The diagnosis requires two hairy cell markers out of CD103, CD123, CD25, and CD11c to be positive. The
HCL variant (
HCL-v) has a different IPT profile with negative CD25 in most cases.
OBJECTIVE: The aim was to study the hematological and IPT of classical
HCL and
HCL variants.
METHODS: This cross-sectional study included all the cases of HCL diagnosed over a retrospective period of eight years from 1st January 2015 to 31st December 2022 in a tertiary care hospital in north India. The patients included in the study were those for whom immunophenotyping; that is, flow cytometry and/or immunohistochemistry (IHC) were done for diagnosis. Bone marrow slides, IHC slides, and flow cytometric IPTs were reviewed.
RESULTS: The study included 13 patients who were diagnosed to have HCL, of which 12 were classical
HCL and one was
HCL-variant (
HCL-v). Among classical HCL, IPT was done by flow cytometry in 10 patients, while in two patients, it was done by IHC. CD19, CD20, and CD22 were positive in all patients of classical HCL (10/10, 10/10, and 5/5, respectively), while CD123, CD103, CD25, and CD11C were positive in 100%, 89%, 80%, and 100% cases, respectively. One patient of
HCL-v had CD103 and CD123 positive, while CD25 and CD123 were negative.
CONCLUSIONS: The diagnosis of
HCL requires a multipronged approach. The use of clinical features, morphology, and immunophenotyping combined with ancillary techniques provides higher diagnostic accuracy and enables its distinction from other B-cell lymphoproliferative disorders (BCLPDs), leading to better patient management and treatment.