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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.

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。

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