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2-氯脱氧腺苷(克拉屈滨)治疗毛细胞白血病

2-chlorodeoxyadenosine (cladribine) in the treatment of hairy cell leukemia.

作者信息

Yang M H, Ho C H

机构信息

Division of Hematology, Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, R.O.C.

出版信息

Zhonghua Yi Xue Za Zhi (Taipei). 2001 Jan;64(1):54-8.

Abstract

Hairy cell leukemia is an uncommon chronic B cell lymphoproliferative disorder in Taiwan and only sporadic cases have been noted. This disease is often found in middle-aged men with the typical manifestations of pancytopenia and splenomegaly. While tartrate-resistant acid phosphatase (TRAP) stain of the neoplastic cells in peripheral blood smear is positive in 90% cases, bone marrow examination is still necessary for diagnosis. For treatment, splenectomy and interferon have been the standard strategies. Recently, newly-developed purine analogues, 2-chlorodeoxyadenosine (cladribine) and 2'-deoxycorfomycin (pentostatin), were found to achieve long lasting complete remission. Here we reported a 48-year-old men who was found to have splenomegaly and bicytopenia incidentally at routine physical checkup. Although the TRAP staining of peripheral blood showed negative result, hairy cell leukemia was finally diagnosed by bone marrow biopsy. The following splenectomy showed a typical picture of hairy cell leukemia on pathology, but cytopenia recovered only partially after surgery. One course of chemotherapy with cladribine (0.1 mg/kg/day continuous intravenous infusion for 7 days) was given after recovery from major surgery. The white cell and platelet counts normalized after treatment and he carried a stable condition thereafter. Since the disease is rare and purine analogues are newly developed, there has been no report on using 2-clorodeoxy-adenosine against hairy cell leukemia in our country. Here we suggest that newly developed purine analogues such as cladribine are the first choice of treatment in cases of hairy cell leukemia.

摘要

毛细胞白血病在台湾是一种罕见的慢性B细胞淋巴增殖性疾病,仅有散发病例报道。这种疾病常见于中年男性,典型表现为全血细胞减少和脾肿大。虽然外周血涂片肿瘤细胞的抗酒石酸酸性磷酸酶(TRAP)染色在90%的病例中呈阳性,但骨髓检查对于诊断仍很有必要。治疗方面,脾切除术和干扰素一直是标准治疗策略。最近,新开发的嘌呤类似物,2-氯脱氧腺苷(克拉屈滨)和2'-脱氧柯福霉素(喷司他丁),被发现可实现持久的完全缓解。在此我们报告一名48岁男性,在常规体检时偶然发现脾肿大和血细胞减少。尽管外周血TRAP染色结果为阴性,但最终通过骨髓活检诊断为毛细胞白血病。随后的脾切除术病理显示为典型的毛细胞白血病表现,但术后血细胞减少仅部分恢复。在大手术恢复后给予一个疗程的克拉屈滨化疗(0.1mg/kg/天持续静脉输注7天)。治疗后白细胞和血小板计数恢复正常,此后病情稳定。由于该疾病罕见且嘌呤类似物是新开发的,我国尚无使用2-氯脱氧腺苷治疗毛细胞白血病的报道。在此我们建议,新开发的嘌呤类似物如克拉屈滨是毛细胞白血病病例的首选治疗药物。

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