Gollard R, Lee T C, Piro L D, Saven A
Division of Hematology/Oncology, Ida M. and Cecil H. Green Cancer Center, Scripps Clinic and Research Foundation, La Jolla, California, USA.
Drugs. 1995 Jun;49(6):921-31. doi: 10.2165/00003495-199549060-00006.
Hairy cell leukaemia is an uncommon B cell chronic lymphoproliferative disorder characterised by circulating lymphocytes displaying prominent cytoplasmic projections. Therapy is initiated for severe cytopenias or recurrent infections. Splenectomy, the first standard treatment, is now less commonly used as primary treatment. Interferon-alpha (IFN alpha) induces partial responses in most patients but complete responses in only a few. Adverse effects from IFN alpha are common but not life-threatening. The ability of two newer purine analogues, pentostatin (2'-deoxycoformycin) and cladribine (2-chlorodeoxyadenosine), to induce long-lasting complete remissions in the majority of patients has revolutionised the treatment of this disease. Cladribine is emerging as the treatment of choice because of its favourable toxicity profile, brief duration of treatment, high percentage of unmaintained complete remissions and low incidence of relapse.
毛细胞白血病是一种罕见的B细胞慢性淋巴细胞增殖性疾病，其特征是循环淋巴细胞显示出明显的胞质突起。对于严重血细胞减少或反复感染的患者开始进行治疗。脾切除术作为最初的标准治疗方法，现在较少用作主要治疗手段。α干扰素（IFNα）可使大多数患者产生部分缓解，但仅有少数患者能达到完全缓解。IFNα的不良反应常见但不危及生命。两种新型嘌呤类似物喷司他丁（2'-脱氧助间型霉素）和克拉屈滨（2-氯脱氧腺苷）能够使大多数患者获得持久的完全缓解，这彻底改变了该疾病的治疗方式。由于克拉屈滨具有良好的毒性特征、治疗疗程短、未维持完全缓解率高以及复发率低等优点，它正逐渐成为首选治疗药物。
