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对造血发育异常认识的临床贡献:13例难治性贫血患者的长期随访

Clinical contribution to the knowledge of hemopoietic dysplasias: long-term follow-up of 13 patients with refractory anemia.

作者信息

Ricci P, Baccarani M, Zaccaria A, Santucci M A, Tura S

出版信息

Acta Haematol. 1978;60(1):10-20. doi: 10.1159/000207690.

DOI:10.1159/000207690
PMID:97896
Abstract

Refractory anemia (RA) is an hematologic disorder at risk of developing into a malignancy (hemopoietic dysplasia). Information on hemopoietic dysplasias is useful for sharpening the appropriate diagnostic criteria and for the search of an appropriate therapy. Moreover, hemopoietic dysplasias provide an interesting opportunity, in humans, for studying a developing leukemia, or a disease situated at the boundary of leukemia. This paper reports on the evolution of RA in 13 patients followed up more than 2 years, through clinical observation, blood and marrow examination, and karyotype analysis. 4 of these 13 patients developed acute or subacute myeloid leukemia. An additional patient died because of severe thrombocytopenia. In these 5 patients, dyserythropoiesis was accompanied by thrombocytopenia, marrow myeloblastosis, and nonrandom chromosome abnormalities.

摘要

难治性贫血(RA)是一种有发展为恶性肿瘤(造血发育异常)风险的血液系统疾病。有关造血发育异常的信息有助于完善恰当的诊断标准并寻找合适的治疗方法。此外,造血发育异常为人类研究正在发展的白血病或处于白血病边缘的疾病提供了一个有趣的机会。本文报告了13例随访超过2年的RA患者的病情演变情况,通过临床观察、血液和骨髓检查以及核型分析进行研究。这13例患者中有4例发展为急性或亚急性髓系白血病。另有1例患者因严重血小板减少症死亡。在这5例患者中,红细胞生成异常伴有血小板减少、骨髓原始粒细胞增多以及非随机性染色体异常。

相似文献

1
Clinical contribution to the knowledge of hemopoietic dysplasias: long-term follow-up of 13 patients with refractory anemia.对造血发育异常认识的临床贡献:13例难治性贫血患者的长期随访
Acta Haematol. 1978;60(1):10-20. doi: 10.1159/000207690.
2
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[The bone marrow karyotype in refractory anemia and preleukemia].
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[The 5q syndrome--a chromosome aberration with characteristic hematologic picture: preleukemia? Literature review and description of a personal case].
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Detection of cytochemical and morphological anomalies in 'preleukemia'.“白血病前期”细胞化学及形态学异常的检测
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Comparison of long-term outcomes between children with aplastic anemia and refractory cytopenia of childhood who received immunosuppressive therapy with antithymocyte globulin and cyclosporine.接受抗胸腺细胞球蛋白和环孢素免疫抑制治疗的再生障碍性贫血患儿与儿童难治性血细胞减少症患儿的长期结局比较。
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Refractory anemia with excess of blast cells: prognostic factors and effect of treatment with androgens or cytosine arabinoside. Results of a prospective trial in 58 patients. Cooperative Group for the Study of Aplastic and Refractory Anemias.伴有原始细胞增多的难治性贫血:预后因素及雄激素或阿糖胞苷治疗的效果。58例患者的前瞻性试验结果。再生障碍性贫血和难治性贫血研究协作组
Cancer. 1979 Dec;44(6):1976-82. doi: 10.1002/1097-0142(197912)44:6<1976::aid-cncr2820440603>3.0.co;2-#.

引用本文的文献

1
Refractory anaemia.难治性贫血
Br Med J. 1980 Aug 23;281(6239):528-9.