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[慢性髓系白血病]

[Chronic myeloid leukemia].

作者信息

Hehlmann R

机构信息

III. Medizinische Klinik, Klinikum Mannheim, Universität Heidelberg, Mannheim.

出版信息

Ther Umsch. 1996 Feb;53(2):82-7.

PMID:8629268
Abstract

The chronic myelogenous leukemia [CML] is a clonal disease of hematopoietic stem cells with unknown etiology. The incidence is around 2/100,000/year, the median age at diagnosis about 47 years. The course of CML is characterized by a chronic phase with few symptoms and good therapeutic response of about 4 to 5 years duration and by transition to a prognostically unfavourable blast phase of about 3 months duration. Therapy of choice, at present, is early allogenous bone marrow transplantation [BMT], which is curative in 40 to 80% of transplanted cases. In patients below 55 years, a donor search should be started at the earliest possible time after diagnosis. Drug therapy of choice are interferon alpha [IFN] and hydroxyurea, which are both superior to busulfan with regard to duration of chronic phase and survival. Complete cytogenetic remissions are observed in 5 to 9% of IFN-treated patients in randomized studies, but virtually all remain positive for bcr/abl by PCR. Whether and in how far IFN is superior to hydroxyurea appears, at least in part, to depend on the treatment intensity with hydroxyurea and on patients characteristics. In analyzing median survival times, the risk profiles of the patients have to be considered. In the future, intensive chemotherapy with or without autografting might play an important role in the therapy of chronic-phase CML. Forthcoming trials have to consider both, conventional and new experimental treatment modalities. An example is the treatment strategy of the ongoing randomized study of the German CML Study Group which compares allogenous BMT with the best available drug therapy and, in addition, analyses the influence of intensified drug therapy on survival.

摘要

慢性粒细胞白血病(CML)是一种造血干细胞的克隆性疾病,病因不明。发病率约为每年2/100,000,诊断时的中位年龄约为47岁。CML的病程特点是慢性期症状较少,治疗反应良好,持续约4至5年,然后转变为预后不良的急变期,持续约3个月。目前的首选治疗方法是早期异基因骨髓移植(BMT),在40%至80%的移植病例中可治愈。对于55岁以下的患者,诊断后应尽早开始寻找供体。首选的药物治疗是干扰素α(IFN)和羟基脲,在慢性期持续时间和生存率方面,两者均优于白消安。在随机研究中,5%至9%接受IFN治疗的患者可观察到完全细胞遗传学缓解,但通过聚合酶链反应(PCR)检测,几乎所有患者的bcr/abl仍为阳性。IFN是否优于羟基脲以及在多大程度上优于羟基脲,至少部分取决于羟基脲的治疗强度和患者特征。在分析中位生存时间时,必须考虑患者的风险特征。未来,强化化疗(无论是否进行自体移植)可能在慢性期CML的治疗中发挥重要作用。即将进行的试验必须同时考虑传统和新的实验性治疗方式。德国CML研究组正在进行的随机研究的治疗策略就是一个例子,该研究比较了异基因BMT与最佳可用药物治疗,并分析了强化药物治疗对生存的影响。

相似文献

1
[Chronic myeloid leukemia].[慢性髓系白血病]
Ther Umsch. 1996 Feb;53(2):82-7.
2
Multicenter prospective study of interferon-alpha and conventional chemotherapy versus bone marrow transplantation for newly diagnosed patients with chronic myelogenous leukemia. Kouseisho Leukemia Study Group.α干扰素与传统化疗对比骨髓移植治疗新诊断慢性粒细胞白血病患者的多中心前瞻性研究。厚生省白血病研究组
Int J Hematol. 2000 Aug;72(2):229-36.
3
Prior treatment with alpha-interferon does not adversely affect the outcome of allogeneic BMT in chronic phase chronic myeloid leukemia.在慢性期慢性髓性白血病中,先前使用α-干扰素治疗不会对异基因骨髓移植的结果产生不利影响。
Haematologica. 1998 Mar;83(3):231-6.
4
Use of granulocyte-macrophage colony-stimulating factor (GM-CSF) in combination with hydroxyurea as post-transplant therapy in chronic myelogenous leukemia patients autografted with unmanipulated hematopoietic cells.在慢性粒细胞白血病患者接受未处理造血细胞自体移植后,使用粒细胞巨噬细胞集落刺激因子(GM-CSF)联合羟基脲作为移植后治疗方法。
Haematologica. 1997 May-Jun;82(3):291-6.
5
[Treatment of chronic myeloid leukemia using alpha-interferon and hydroxyurea. Study of 30 cases].[使用α-干扰素和羟基脲治疗慢性粒细胞白血病。30例研究]
Sangre (Barc). 1994 Jun;39(3):183-6.
6
High-dose chemo-radiotherapy followed by autologous Philadelphia chromosome-negative blood progenitor cell transplantation in patients with chronic myelogenous leukemia.慢性粒细胞白血病患者接受高剂量化疗放疗后进行自体费城染色体阴性血液祖细胞移植。
Bone Marrow Transplant. 1996 Feb;17(2):201-5.
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Randomized comparison of interferon alpha and hydroxyurea with hydroxyurea monotherapy in chronic myeloid leukemia (CML-study II): prolongation of survival by the combination of interferon alpha and hydroxyurea.干扰素α与羟基脲联合治疗与羟基脲单药治疗慢性粒细胞白血病的随机对照研究(CML研究II):干扰素α与羟基脲联合治疗可延长生存期
Leukemia. 2003 Aug;17(8):1529-37. doi: 10.1038/sj.leu.2403006.
8
Chemotherapy and bone marrow transplantation in the treatment of chronic myelogenous leukemia.化疗和骨髓移植治疗慢性粒细胞白血病。
Semin Oncol. 1994 Dec;21(6 Suppl 14):8-13.
9
[Interferon alpha--the drug of choice for patients with chronic myeloid leukemia].
Cas Lek Cesk. 1998 Sep 21;137(18):552-6.
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[Prognosis of children with chronic myeloid leukemia: a retrospective analysis of 75 patients].[慢性髓性白血病患儿的预后:75例患者的回顾性分析]
Klin Padiatr. 1996 Jul-Aug;208(4):236-41. doi: 10.1055/s-2008-1046479.