Fanta Paul Timothy, Saven Alan
Scripps Clinic, La Jolla, CA, USA.
Cancer Treat Res. 2008;142:193-209. doi: 10.1007/978-0-387-73744-7_8.
Hairy cell leukemia is an indolent B-cell non-Hodgkin's lymphoma with a characteristic presentation of pancytopenia, splenomegaly, and circulating hairy cells. An immunophenotypic pattern of CD11c, CD25, and CD103 expression. TRAP staining, reticulin deposition, and morphology of bone marrow and circulating cells help establish the diagnosis. Although up to 10% of patients might not require systemic treatment, for the vast majority effective treatments are available with the purine-nucleoside analogues cladribine and pentostatin. Cladribine is considered the drug of choice in the first-line setting due to the very high complete remission rate and prolonged duration of response following a single 7-day infusion. Cladribine and pentostatin both have unique but different mechanisms of action, with a lack of cross-resistance between them, which might be exploited in the relapsed or refractory disease setting. Therapy for relapsed and refractory patients also includes novel biologic agents as well as splenectomy. Despite the effective treatment options, the prospect of cure remains elusive due to the frequent presence of MRD even in complete responders. Future studies employing combination therapies targeting the eradication of MRD will hopefully improve relapse-free survivals as well as overall survival, and might even offer the prospect of cure.
毛细胞白血病是一种惰性B细胞非霍奇金淋巴瘤，具有全血细胞减少、脾肿大和循环毛细胞的特征性表现。存在CD11c、CD25和CD103表达的免疫表型模式。TRAP染色、网状纤维沉积以及骨髓和循环细胞的形态有助于确诊。尽管高达10%的患者可能不需要全身治疗，但对于绝大多数患者而言，可使用嘌呤核苷类似物克拉屈滨和喷司他丁进行有效治疗。由于单次7天输注后完全缓解率非常高且缓解持续时间延长，克拉屈滨被认为是一线治疗的首选药物。克拉屈滨和喷司他丁都有独特但不同的作用机制，它们之间缺乏交叉耐药性，这在复发或难治性疾病的治疗中可能会被利用。复发和难治性患者的治疗还包括新型生物制剂以及脾切除术。尽管有有效的治疗选择，但由于即使是完全缓解者也经常存在微小残留病，治愈的前景仍然渺茫。未来采用针对根除微小残留病的联合疗法的研究有望提高无复发生存率以及总生存率，甚至可能带来治愈的前景。
