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获得性再生障碍性贫血的预后因素。352例病例研究。

Prognostic factors in acquired aplastic anemia. A study of 352 cases.

作者信息

Najean Y, Pecking A

出版信息

Am J Med. 1979 Oct;67(4):564-71. doi: 10.1016/0002-9343(79)90226-2.

Abstract

The prognostic factors of short- and long-term survival have been studied in 352 patients with aplastic anemia of all grades of severity. This group was homogeneous with regard to the clinical and laboratory survey, and the treatment used [high-dose androgen therapy]. The "hierarchy" of the individual prognostic parameters has been established: current severe infection, granulocyte count, percentage of the nonmyeloid cells on the bone marrow slides, platelet count, reticulocyte count, 59Fe utilization, and stromal disorganization on the bone marrow biopsy specimen. As these parameters are interrelated, a multiparametric analysis enables us to define groups of patients with different short-term evolution and to derive a prognostic index from these data. The use of such an index, however, allows a correct prediction in only 73 per cent of the cases, better in the milder than in the more severe cases. It is possible that the short-term evolutive tendency (improvement or worsening during the first six weeks of therapy) may contribute supplementary information useful for prognosis and the choice of treatment. After the first three months critical period, the mortality rate no longer depends on the initial severity of the disease but exclusively on the clinical and hematologic improvement. Thus, comparing the hematologic data obtained initially and after three months of androgen therapy allows us to correctly predict the long-term evolution.

摘要

对352例不同严重程度的再生障碍性贫血患者的短期和长期生存预后因素进行了研究。该组患者在临床和实验室检查以及所采用的治疗方法(高剂量雄激素疗法)方面具有同质性。已确定了个体预后参数的“层级”:当前严重感染、粒细胞计数、骨髓涂片上非髓细胞百分比、血小板计数、网织红细胞计数、59Fe利用率以及骨髓活检标本中的基质紊乱。由于这些参数相互关联,多参数分析使我们能够定义具有不同短期病程的患者群体,并从这些数据中得出一个预后指数。然而,使用这样一个指数仅能在73%的病例中做出正确预测,轻症病例的预测效果优于重症病例。短期病程趋势(治疗前六周内病情改善或恶化)可能会提供有助于预后和治疗选择的补充信息。在最初三个月的关键期过后,死亡率不再取决于疾病的初始严重程度,而是完全取决于临床和血液学改善情况。因此,比较最初获得的血液学数据与雄激素治疗三个月后的数据,能够使我们正确预测长期病程。

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