Division of Hematology/Oncology, Scripps Clinic, La Jolla, CA 92037, USA.
Leuk Lymphoma. 2011 Jun;52 Suppl 2:29-33. doi: 10.3109/10428194.2011.569621. Epub 2011 Apr 19.
Hairy cell leukemia (HCL) is an indolent, mature B-cell lymphoproliferative malignancy that clinically manifests as pancytopenia and fibrosis of the bone marrow, ultimately leading to recurrent infections and splenomegaly. This article reviews the extensive experience at Scripps Clinic with cladribine (2-chlorodeoxyadenosine; 2-CdA) in HCL. It is currently recommended as first-line treatment in HCL, because of its proven efficacy with associated low toxicity profile and brevity of treatment duration, with the majority of responses being complete (CRs). This was achieved after only a single 7-day infusion. We also discuss the success of cladribine in patients with relapsed and refractory HCL and potential approaches to eradicating minimal residual disease (MRD). MRD is thought to predict for future relapses. The eradication of MRD warrants continued study in order to further improve disease-free survival, which may translate into ultimately curing patients with HCL.
毛细胞白血病（HCL）是一种惰性的成熟 B 细胞淋巴增殖性恶性肿瘤，临床上表现为全血细胞减少和骨髓纤维化，最终导致反复感染和脾肿大。本文回顾了 Scripps 诊所使用克拉屈滨（2-氯脱氧腺苷；2-CdA）治疗 HCL 的广泛经验。由于其疗效确切，毒性低，治疗时间短，大多数反应为完全缓解（CR），目前被推荐作为 HCL 的一线治疗药物。这在仅单次 7 天输注后即可实现。我们还讨论了克拉屈滨在复发性和难治性 HCL 患者中的成功应用，以及消除微小残留病（MRD）的潜在方法。MRD 被认为可预测未来的复发。消除 MRD 需要进一步研究，以进一步提高无病生存率，这可能最终使 HCL 患者得到治愈。
