PDF(Pubmed)
Abstract：
Hairy cell leukemia (HCL) represents 2% of all leukemia cases, with men aged above 55 years being the most affected. The most common symptoms of this type of leukemia include splenomegaly, monocytopenia, and neutropenia. In the basic blood count examination, leukopenia with monocytopenia and granulocytopenia, as well as aplastic anemia and/or thrombocytopenia occur. The mutation of β-rapidly accelerated fibrosarcoma (BRAF) proto-oncogene, which can be found in nearly 100% of patients, is an important feature of HCL. Immunophenotypic analysis of the HCL cells reveals high expression of B-lineage antigens, including CD19, CD20, and CD22. Additionally, CD11c, CD25, CD103, and CD123 belong to specific markers of HCL. Lactate dehydrogenase activity and β-2-microglobulin concentration are also important in the patient\'s assessment. The differential diagnosis between HCL, hairy cell leukemia variant (HCL-V) and splenic marginal zone lymphoma (SMZL) is of first importance. Currently, the main treatment for HCL involves the use of purine analogues, excluding pregnant women, individuals with severe infections, and those with relapsing HCL.
摘要：
毛状细胞白血病（HCL）占所有白血病病例的2％，55岁以上的男性受影响最大。这类白血病最常见的症状包括脾肿大，单核细胞减少症,和中性粒细胞减少症.在基础血细胞计数检查中，白细胞减少伴单核细胞减少症和粒细胞减少症，以及再生障碍性贫血和/或血小板减少症的发生。β-快速加速纤维肉瘤(BRAF)原癌基因的突变,几乎100%的病人都能发现,是HCL的重要特征。HCL细胞的免疫表型分析揭示了B系抗原的高表达，包括CD19、CD20和CD22。此外,CD11c,CD25、CD103和CD123属于HCL的特异性标志物。乳酸脱氢酶活性和β-2-微球蛋白浓度在患者评估中也很重要。HCL之间的鉴别诊断，毛细胞白血病变异型（HCL-V）和脾边缘区淋巴瘤（SMZL）至关重要。目前,HCL的主要治疗方法包括使用嘌呤类似物，不包括孕妇,患有严重感染的人，和那些复发的盐酸。
