脱氧助间型霉素成功治疗毛细胞白血病

[Hairy cell leukemia successfully treated with deoxycoformycin].

作者信息

Matsuda A, Jinnai I, Mizuno H, Sakata T, Kusumoto S, Kayano H, Takeuchi H, Bessho M, Saito M, Katayama I

机构信息

First Department of Internal Medicine, Saitama Medical School.

出版信息

Rinsho Ketsueki. 1992 Nov;33(11):1685-90.

PMID:1469784

Abstract

A 45-year-old male was hospitalized on September 2, 1989 with chief complaints of general fatigue and fever. Physical examination revealed hepatomegaly and massive splenomegaly. Laboratory tests on admission showed Hb of 7.5g/dl, PLT 4.8 x 10(4)/microliters and WBC 9,610/microliters with 81% hairy cells. Bone marrow aspirate demonstrated 55.1% hairy cells and moderate myelofibrosis. Cytochemically, hairy cells were positive for tartrate-resistant acid phosphatase (TRAP). Surface markers were SmIg G+ A+ kappa +, CD11b+, CD11c+, CD19+, CD20+, CD21-, CD25+, HC2+, HLA-DR+. From these findings, a diagnosis of hairy cell leukemia (HCL) was made. After administration of deoxycoformycin (DCF) at a dose of 5.0mg/m2 1-2 times monthly, splenomegaly disappeared, as did hairy cells from the peripheral blood. Hematological level returned to within normal range except for the presence of 1.2% hairy cells and mild myelofibrosis in bone marrow aspirates. DCF has so far been effective for this patient. While DCF has been reported to be effective in the treatment of HCL in the West, it has not been determined in Japanese patients with HCL, who have different hematologic features from those of HCL patients in the West.

摘要

一名45岁男性于1989年9月2日住院，主要症状为全身乏力和发热。体格检查发现肝脏肿大和巨脾。入院时实验室检查显示血红蛋白（Hb）为7.5g/dl，血小板（PLT）4.8×10⁴/微升，白细胞（WBC）9610/微升，其中81%为毛细胞。骨髓穿刺显示55.1%为毛细胞，伴有中度骨髓纤维化。细胞化学检查显示，毛细胞对耐酒石酸酸性磷酸酶（TRAP）呈阳性。表面标志物为SmIg G⁺ A⁺ κ⁺、CD11b⁺、CD11c⁺、CD19⁺、CD20⁺、CD21⁻、CD25⁺、HC2⁺、HLA-DR⁺。根据这些发现，诊断为毛细胞白血病（HCL）。每月1 - 2次给予剂量为5.0mg/m²的脱氧助间型霉素（DCF）后，脾肿大消失，外周血中的毛细胞也消失。除骨髓穿刺中有1.2%的毛细胞和轻度骨髓纤维化外，血液学指标恢复至正常范围。迄今为止，DCF对该患者有效。虽然在西方已报道DCF对HCL治疗有效，但在日本HCL患者中尚未确定，日本HCL患者的血液学特征与西方HCL患者不同。