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获得性再生障碍性贫血的预后

Prognosis in acquired aplastic anemia.

作者信息

Hörmann A, Berchthold W, Rhyner K, Wiedemann-Sigg C, Gmür J

出版信息

Acta Haematol. 1984;71(2):81-9. doi: 10.1159/000206562.

Abstract

To study prognostic factors 38 consecutive patients with aplastic anemia were evaluated until death or over a follow-up period of at least 4 years. A prognostic formula was devised by multivariate analysis for prediction of short-term survivors at diagnosis of aplastic anemia: Using the initial percentage of nonmyeloid cells in the marrow aspirate and the neutrophil count in peripheral blood, survival of less than 4 months is predicted with a probability of 89% (sensitivity 80%). The lowest blood counts during the course of the disease are useful to predict the outcome in more chronic courses of aplastic anemia: A reticulocytopenia below 5 X 10(9)/1, a neutropenia below 0.1 X 10(9)/1 or a thrombocytopenia below 5 X 10(9)/1 all resulted in an ultimate mortality of over 90%. However, if none of these threshold values were reached, a 4 year survival of 71% was observed. Thus, 5 X 10(9) reticulocytes/1, 0.1 X 10(9) neutrophils/1 and 5 X 10(9) platelets/1 form a group of risk factors to predict a fatal or favorable outcome throughout the course of the disease.

摘要

为研究预后因素,对38例再生障碍性贫血患者进行了连续评估,直至死亡或随访至少4年。通过多变量分析设计了一个预后公式,用于预测再生障碍性贫血诊断时的短期存活者:利用骨髓穿刺液中非髓细胞的初始百分比和外周血中性粒细胞计数,预测生存期少于4个月的概率为89%(敏感性80%)。疾病过程中的最低血细胞计数有助于预测再生障碍性贫血更慢性病程的预后:网织红细胞减少低于5×10⁹/L、中性粒细胞减少低于0.1×10⁹/L或血小板减少低于5×10⁹/L,最终死亡率均超过90%。然而,如果未达到这些阈值中的任何一个,则观察到4年生存率为71%。因此,5×10⁹/L网织红细胞、0.1×10⁹/L中性粒细胞和5×10⁹/L血小板构成一组风险因素,可预测疾病整个病程的致命或良好预后。

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