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目的探讨毛细胞白血病(HCL)的临床病理学特征及免疫表型特点。方法回顾性分析12例HCL患者的临床及实验室检查资料,收集患者的年龄、性别、临床表现、病情、病程及骨髓形态学、骨髓活检组织学、流式细胞学检查结果,并进行分析。结果 12例HCL患者中,男9例、女3例、年龄38~71岁、中位年龄49岁。临床表现主要为乏力、贫血。12例HCL患者中,贫血11例,发热2例,脾大12例,淋巴结肿大4例,肝大4例。RBC 2.14~3.97×1012/L,WBC 2.51~17.33×109/L,PLT 67.83~128.02×109/L。骨髓细胞形态学检查发现有典型毛细胞者8例。免疫表型分析结果显示,均同时表达成熟B细胞表面抗原(CD19、CD20和CD79a),同时CD11c、CD25、CD103阳性者10例。行骨髓病理学检查11例,其中毛细胞广泛浸润9例,毛细胞呈“油煎蛋”样外观以及网硬蛋白纤维显著增多。结论 HCL主要见于中老年男性,临床表现主要为乏力和疲劳,体征主要有脾大。血细胞减少,外周血及骨髓形态学检查及骨髓病理学检查发现毛细胞为诊断依据。毛细胞免疫表型主要表达B细胞相关抗原CD19、CD20、CD22、CD79a和FMC7,CD11c、CD25、CD103及CD123。
Objective To investigate the clinicopathological and immunophenotypic features of hairy cell leukemia (HCL). Methods The clinical and laboratory data of 12 patients with HCL were retrospectively analyzed. The patients’ age, gender, clinical manifestation, disease, course of disease, bone marrow morphology, bone marrow biopsy histology and flow cytometry were analyzed retrospectively. Results Among the 12 HCL patients, 9 were males and 3 were females, aged 38-71 years, with a median age of 49 years. The main clinical manifestations of fatigue, anemia. Of the 12 HCL patients, 11 were anemic, 2 were fever, 12 were splenomegaly, 4 were lymphadenopathy and 4 were hepatocellular carcinoma. RBC 2.14 ~ 3.97 × 1012 / L, WBC 2.51 ~ 17.33 × 109 / L, PLT 67.83 ~ 128.02 × 109 / L. Bone marrow cell morphological examination found that there are 8 cases of typical hair cells. The results of immunophenotyping showed that all of the mature B cell surface antigens (CD19, CD20 and CD79a) were expressed simultaneously, while 10 cases were positive for CD11c, CD25 and CD103. Pathological examination of bone marrow in 11 cases, including extensive infiltration of hair cells in 9 cases, the hair cells were “fried egg” -like appearance and spindle protein fibers increased significantly. Conclusion HCL is mainly seen in middle-aged and elderly men, the main clinical manifestations of fatigue and fatigue, the main signs of splenomegaly. Cytopenia, peripheral blood and bone marrow morphology and bone marrow pathology examination found that hair cells for the diagnosis. Hair cell immunophenotypes mainly express B cell-associated antigens CD19, CD20, CD22, CD79a and FMC7, CD11c, CD25, CD103 and CD123.