Quigley Michael M, Bethel Kelly J, Sharpe Robert W, Saven Alan
Division of Hematopathology, Scripps Clinic Laboratories, Scripps Clinic, La Jolla, California 92037, USA.
Am J Hematol. 2003 Dec;74(4):227-30. doi: 10.1002/ajh.10428.
Hairy cell leukemia (HCL) is a rare chronic B-cell lymphoproliferative disorder characterized by splenomegaly, pancytopenia, and circulating atypical lymphocytes with circumferential cytoplasmic projections. Although uncommon, HCL cases refractory to standard therapy occur, and effective alternatives are limited. There is evolving literature supporting monoclonal antibody therapy in the treatment of B-cell lymphoid malignancies, including anti-CD52 (Campath-1H, alemtuzumab). We have examined nine cases of HCL and one case of HCL variant by flow cytometry for CD52 expression. All cases expressed CD52 antigen in 92-100% of the malignant cells. The demonstration of CD52 antigen expression on HCL cells provides the rationale for the use of alemtuzumab in refractory HCL.
毛细胞白血病(HCL)是一种罕见的慢性B细胞淋巴增殖性疾病,其特征为脾肿大、全血细胞减少以及循环中出现带有周边胞质突起的非典型淋巴细胞。尽管不常见,但存在对标准治疗难治的HCL病例,且有效的替代治疗方法有限。有不断发展的文献支持单克隆抗体疗法用于治疗B细胞淋巴恶性肿瘤,包括抗CD52(Campath-1H,阿仑单抗)。我们通过流式细胞术检测了9例HCL病例和1例HCL变异型病例的CD52表达情况。所有病例中92%-100%的恶性细胞表达CD52抗原。HCL细胞上CD52抗原表达的证实为阿仑单抗用于难治性HCL提供了理论依据。