Tetreault S A, Robbins B A, Saven A
Division of Hematology and Oncology, Ida M. and Cecil H. Green Cancer Center, Scripps Clinic, La Jolla, California 92037, USA.
Leuk Lymphoma. 1999 Oct;35(3-4):347-54. doi: 10.3109/10428199909145739.
Hairy cell leukemia-variant (HCL-V) is an extremely rare chronic B-cell lymphoproliferative disorder clinically and morphologically distinct from classic hairy cell leukemia (HCL). HCL-V is thought to represent a hybrid between prolymphocytic leukemia and HCL, the nucleus more closely resembling a prolymphocyte and the cytoplasm a hairy cell. The clinical course of HCL-V is aggressive with short survivals. Since single courses of cladribine have profound activity in HCL, inducing durable complete responses in 91% of patients, we administered cladribine to 4 patients with HCL-V over a 7-year period. During this time interval 357 patients with classic HCL received cladribine at Scripps Clinic. Each patient received cladribine at 0.1 mg/kg per day by continuous intravenous infusion for 7 days, repeated at 28-day intervals depending on response status. The 4 patients ranged in age from 28 to 70. Two presented with B-symptoms, 1 had peripheral adenopathy, and all 4 displayed massive splenomegaly. Peripheral blood counts were notable for lymphocytosis associated with mild anemia and thrombocytopenia. Only 1 of the 4 patients had received prior treatment. Peripheral blood immunophenotypic analysis revealed monoclonal B cells with expression of CD11c in 3 patients, lack of CD25 expression in 3 patients and expression of CD103 in all but 1 patient. The number of cladribine courses administered ranged from two to five. Of these 4 patients, 1 (25%) achieved a complete response and 2 (50%) partial responses, for an overall response rate of 75%. Three patients underwent splenectomy after cladribine. Cladribine is an active agent in HCL-V albeit with a lower response rate than in classic HCL. The role of other treatment modalities, such as splenectomy, interferon-alpha, and 2'-deoxycoformycin, alone or in combination with cladribine awaits further evaluation.
变异型毛细胞白血病（HCL-V）是一种极为罕见的慢性B细胞淋巴增殖性疾病，在临床和形态学上与经典毛细胞白血病（HCL）不同。HCL-V被认为是原淋巴细胞白血病和HCL之间的一种混合类型，其细胞核更类似于原淋巴细胞，而细胞质则类似毛细胞。HCL-V的临床病程进展迅速，生存期短。由于克拉屈滨单疗程对HCL具有显著活性，可使91%的患者获得持久的完全缓解，因此我们在7年时间里对4例HCL-V患者使用了克拉屈滨。在此期间，357例经典HCL患者在斯克里普斯诊所接受了克拉屈滨治疗。每位患者每天以0.1mg/kg的剂量持续静脉输注克拉屈滨7天，根据反应情况每28天重复一次。这4例患者年龄在28至70岁之间。2例有B症状，1例有外周淋巴结病，所有4例均有巨大脾肿大。外周血细胞计数显示淋巴细胞增多，并伴有轻度贫血和血小板减少。4例患者中只有1例曾接受过先前治疗。外周血免疫表型分析显示，3例患者的单克隆B细胞表达CD11c，3例患者缺乏CD25表达，除1例患者外其余患者均表达CD103。克拉屈滨的疗程数为2至5个。这4例患者中，1例（25%）获得完全缓解，2例（50%）获得部分缓解，总缓解率为75%。3例患者在使用克拉屈滨后接受了脾切除术。克拉屈滨在HCL-V中是一种有效药物，尽管其缓解率低于经典HCL。其他治疗方式，如脾切除术、α干扰素和2'-脱氧助间型霉素，单独或与克拉屈滨联合使用的作用有待进一步评估。
