Abstract：
Hairy cell leukemia (HCL) and HCL-like disorders, including HCL variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogenous group of mature lymphoid B-cell disorders characterized by the identification of hairy cells, a specific genetic profile, a different clinical course and the need for appropriate treatment.
Diagnosis of HCL is based on morphological evidence of hairy cells, an HCL immunologic score of 3 or 4 based on the CD11c, CD103, CD123, and CD25 expression, the trephine biopsy which makes it possible to specify the degree of tumoral bone marrow infiltration and the presence of BRAFV600E somatic mutation.
Progression of patients with HCL is based on a large splenomegaly, leukocytosis, a high number of hairy cells in the peripheral blood, and the immunoglobulin heavy chain variable region gene mutational status. VH4-34 positive HCL cases are associated with a poor prognosis, as well as HCL with TP53 mutations and HCL-V.
Patients should be treated only if HCL is symptomatic. Chemotherapy with risk-adapted therapy purine analogs (PNAs) are indicated in first-line HCL patients. The use of chemo-immunotherapy combining cladribine (CDA) and rituximab (R) represents an increasingly used therapeutic approach. Management of relapsed/refractory disease is based on the use of BRAF inhibitors (BRAFi) plus R, MEK inhibitors (MEKi), recombinant immunoconjugates targeting CD22, Bruton tyrosine kinase inhibitors (BTKi), and Bcl-2 inhibitors (Bcl-2i). However, the optimal sequence of the different treatments remains to be determined.
Diagnosis of HCL is based on morphological evidence of hairy cells, an HCL immunologic score of 3 or 4 based on the CD11c, CD103, CD123, and CD25 expression, the trephine biopsy which makes it possible to specify the degree of tumoral bone marrow infiltration and the presence of BRAFV600E somatic mutation.
Progression of patients with HCL is based on a large splenomegaly, leukocytosis, a high number of hairy cells in the peripheral blood, and the immunoglobulin heavy chain variable region gene mutational status. VH4-34 positive HCL cases are associated with a poor prognosis, as well as HCL with TP53 mutations and HCL-V.
Patients should be treated only if HCL is symptomatic. Chemotherapy with risk-adapted therapy purine analogs (PNAs) are indicated in first-line HCL patients. The use of chemo-immunotherapy combining cladribine (CDA) and rituximab (R) represents an increasingly used therapeutic approach. Management of relapsed/refractory disease is based on the use of BRAF inhibitors (BRAFi) plus R, MEK inhibitors (MEKi), recombinant immunoconjugates targeting CD22, Bruton tyrosine kinase inhibitors (BTKi), and Bcl-2 inhibitors (Bcl-2i). However, the optimal sequence of the different treatments remains to be determined.
摘要：
背景：毛状细胞白血病（HCL）和HCL样疾病，包括HCL变异体（HCL-V）和脾弥漫性红髓淋巴瘤（SDRPL），是一组非常异质的成熟淋巴B细胞疾病，其特征是毛细胞的鉴定，一个特定的遗传特征,不同的临床过程和需要适当的治疗。
方法：HCL的诊断基于毛细胞的形态学证据，基于CD11c的HCL免疫学评分为3或4，CD103、CD123和CD25表达，环钻活检可以确定肿瘤骨髓浸润的程度和BRAFV600E体细胞突变的存在。
方法：HCL患者的进展是基于脾肿大，白细胞增多,外周血中大量的毛细胞，和免疫球蛋白重链可变区基因突变状态。VH4-34阳性HCL病例与不良预后相关，以及具有TP53突变和HCL-V的HCL
方法：只有在有症状的HCL患者才应接受治疗。在一线HCL患者中，应使用适应风险的嘌呤类似物（PNA）进行化疗。联合克拉屈滨（CDA）和利妥昔单抗（R）的化学免疫疗法的使用代表了越来越多使用的治疗方法。复发/难治性疾病的管理是基于使用BRAF抑制剂（BRAFi）加R，MEK抑制剂（MEKi），靶向CD22的重组免疫缀合物，布鲁顿酪氨酸激酶抑制剂（BTKi），和Bcl-2抑制剂(Bcl-2i)。然而,不同治疗的最佳顺序仍有待确定。
方法：HCL的诊断基于毛细胞的形态学证据，基于CD11c的HCL免疫学评分为3或4，CD103、CD123和CD25表达，环钻活检可以确定肿瘤骨髓浸润的程度和BRAFV600E体细胞突变的存在。
方法：HCL患者的进展是基于脾肿大，白细胞增多,外周血中大量的毛细胞，和免疫球蛋白重链可变区基因突变状态。VH4-34阳性HCL病例与不良预后相关，以及具有TP53突变和HCL-V的HCL
方法：只有在有症状的HCL患者才应接受治疗。在一线HCL患者中，应使用适应风险的嘌呤类似物（PNA）进行化疗。联合克拉屈滨（CDA）和利妥昔单抗（R）的化学免疫疗法的使用代表了越来越多使用的治疗方法。复发/难治性疾病的管理是基于使用BRAF抑制剂（BRAFi）加R，MEK抑制剂（MEKi），靶向CD22的重组免疫缀合物，布鲁顿酪氨酸激酶抑制剂（BTKi），和Bcl-2抑制剂(Bcl-2i)。然而,不同治疗的最佳顺序仍有待确定。
