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[急性粒细胞白血病伴近四倍体染色体组成,出现在疾病晚期,存活近9年]

[Acute granulocytic leukemia with near-tetraploid chromosome constitution appearing in the late stage of the disease with survival of almost 9 years].

作者信息

Oguma N, Tanaka K, Kamada N, Takimoto Y, Asaoku H, Takemoto M, Kuramoto A

出版信息

Rinsho Ketsueki. 1989 Apr;30(4):502-8.

PMID:2671439
Abstract

A near-tetraploid clone was observed at the 5th relapse in a 34 year-old female with acute granulocytic leukemia. She was referred to Hiroshima University Hospital presenting with palpitation in May 1978. On admission she had a WBC count of 2,900/microliters with 31.5% blasts, platelet count of 2.4 x 10(4)/microliters and hemoglobin of 2.8 g/dl. A bone marrow aspirate showed hypercellular with 88.5% blasts. Most of them were weakly positive for peroxidase reaction. Occasional Auer rod and/or fine azurophilic granules were observed. DCMP therapy (daunorubicin, cytosine arabinoside, 6-mercaptopurine, and prednisone) was started and the first complete remission was achieved in August 1978. Afterward, the 5th relapse occurred in August 1986 and several kinds of drugs were given without effects. She died 8 years and 9 months after the diagnosis. Karyotype prior to treatment showed a normal diploidy without tetraploid mitoses. However, cells with near-tetraploid (mode: 91) were observed predominantly on the 5th relapse sample and almost all mitoses were occupied with near-tetraploidy at the terminal stage. Results of serial cytogenetic and hematologic study showed a close correlation between the incidence of near-tetraploid mitoses and that of very large blast cells. The significance of tetraploidy in previously reported 44 cases including our case was discussed.

摘要

在一名34岁的急性粒细胞白血病女性患者第5次复发时观察到一个近四倍体克隆。1978年5月,她因心悸被转诊至广岛大学医院。入院时,她的白细胞计数为2900/微升,原始细胞占31.5%,血小板计数为2.4×10⁴/微升,血红蛋白为2.8克/分升。骨髓穿刺显示细胞增多,原始细胞占88.5%。其中大多数过氧化物酶反应呈弱阳性。偶尔可见奥氏小体和/或细小嗜天青颗粒。开始采用DCMP疗法(柔红霉素、阿糖胞苷、6-巯基嘌呤和泼尼松),1978年8月首次实现完全缓解。此后,1986年8月发生第5次复发,使用了多种药物均无效。诊断8年9个月后她去世。治疗前的核型显示为正常二倍体,无四倍体有丝分裂。然而,在第5次复发样本中主要观察到近四倍体(众数:91)细胞,在末期几乎所有有丝分裂都为近四倍体。系列细胞遗传学和血液学研究结果显示近四倍体有丝分裂的发生率与非常大的原始细胞的发生率密切相关。讨论了包括我们病例在内的先前报道的44例病例中四倍体的意义。

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