[低剂量阿糖胞苷成功治疗发育不全性混合谱系白血病]

[Hypoplastic mixed lineage leukemia successfully treated with low-dose cytosine arabinoside].

作者信息

Sasaki S, Ueda S, Asakura Y, Yamazaki E, Kato K, Taguchi J, Harano H, Kanamori H, Ogawa K, Otsuka M, Matuszaki M, Mohri H, Okubo T

机构信息

First Department of Internal Medicine, Yokohama City University School of Medicine, Japan.

出版信息

Rinsho Ketsueki. 1996 Mar;37(3):233-8.

PMID:8727348

Abstract

A 54-year-old male was admitted because of dyspnea on exercise. His peripheral blood revealed pancytopenia with severely hypoplastic bone marrow. Bone marrow aspiration showed a marked hypocellular marrow with 62.4% of blast cells. Cytochemical studies showed that peroxidase activity, alpha-nephtyl buthylate esterase activity and PAS reaction were negative, and that only ASD-chroloacetate esterase activity was positive. Surface marker analysis of blast cells showed positive result for CD5, 7, 33 and 34 antigens. The T-cell receptor beta gene was rearranged, but the immunoglobulin H chain gene showed a germ line configuration. Terminal Deoxynucleotydyl Transferase (TdT) was positive, but cellular surface and cytoplasmic immunoglobulin were not recognized. A diagnosis of hypoplastic mixed lineage leukemia was made and treated with low dose cytosine arabinoside, he resulted in complete remission. The relation between hypoplastic leukemia, AML-M0 and mixed lineage leukemia was also discussed.

摘要

一名54岁男性因运动时呼吸困难入院。其外周血显示全血细胞减少，骨髓严重发育不良。骨髓穿刺显示骨髓显著细胞减少，原始细胞占62.4%。细胞化学研究表明，过氧化物酶活性、α-萘丁酸酯酶活性和PAS反应均为阴性，仅ASD-氯乙酸酯酶活性为阳性。原始细胞的表面标志物分析显示CD5、7、33和34抗原呈阳性结果。T细胞受体β基因重排，但免疫球蛋白H链基因呈种系构型。末端脱氧核苷酸转移酶（TdT）呈阳性，但未识别出细胞表面和细胞质免疫球蛋白。诊断为发育不良性混合谱系白血病，并给予小剂量阿糖胞苷治疗，患者完全缓解。还讨论了发育不良性白血病、AML-M0和混合谱系白血病之间的关系。