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[骨髓增生异常综合征的诊断与治疗策略]

[Diagnosis and therapy strategy in myelodysplastic syndromes].

作者信息

Gattermann N, Aul C

机构信息

Abteilung für Hämatologie, Onkologie und klinische Immunologie, Heinrich-Heine-Universität, Düsseldorf.

出版信息

Praxis (Bern 1994). 1996 Jan 16;85(3):39-44.

PMID:8578048
Abstract

Myelodysplastic syndromes (MDS) are diagnosed with increasing frequency in recent years. Their crude annual incidence (ca. 4/100,000), is now about twice that of acute myeloid leukemias (AML). In over 70-year-old patients (age-related incidence more than 20/100,000), MDS rival CLL as the most common hematological neoplasias. Diagnosis of MDS is based on cytological and histological examination of bone marrow specimens, which may show various dysplastic features of hematopoietic precursors and an increased percentage of blast cells. In difficult cases the diagnosis of MDS is supported if cytogenetic investigation reveals an abnormal karyotype. Most patients with MDS (60 bis 80%) die from their bone marrow disorder, with infections, hemorrhages and leukemic transformation being the most frequent causes of death. Treatment must take into account the age and general condition of the individual patient as well as the natural course of his MDS. The comparatively favourable prognosis of patients with early-stage MDS should not be jeopardized by therapies carrying a high risk of treatment-related death. Therefore, treatment is often confined to supportive measures (blood transfusions, antibiotics). Patients with increased percentage of blasts, however, have such a poor prognosis that more aggressive approaches including intensive chemotherapy appear justified. Only very few patients can be treated with allogeneic bone marrow transplantation, which offers a chance for cure.

摘要

近年来,骨髓增生异常综合征(MDS)的诊断频率日益增加。其粗略的年发病率(约4/10万),目前约为急性髓系白血病(AML)的两倍。在70岁以上的患者中(年龄相关发病率超过20/10万),MDS与慢性淋巴细胞白血病(CLL)一样,是最常见的血液系统肿瘤。MDS的诊断基于骨髓标本的细胞学和组织学检查,骨髓标本可能显示造血前体细胞的各种发育异常特征以及原始细胞百分比增加。在疑难病例中,如果细胞遗传学检查显示核型异常,则支持MDS的诊断。大多数MDS患者(60%至80%)死于骨髓疾病,感染、出血和白血病转化是最常见的死亡原因。治疗必须考虑个体患者的年龄和一般状况以及其MDS的自然病程。早期MDS患者相对较好的预后不应因具有高治疗相关死亡风险的治疗而受到损害。因此,治疗通常限于支持性措施(输血、抗生素)。然而,原始细胞百分比增加的患者预后很差,以至于包括强化化疗在内的更积极的治疗方法似乎是合理的。只有极少数患者可以接受异基因骨髓移植,而异基因骨髓移植提供了治愈的机会。

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