Sleijfer D T, Mulder N H, Nieweg H O
Blut. 1981 Feb;42(2):69-78. doi: 10.1007/BF01030028.
In 43 patients with aplastic anaemia we assessed the accuracy of different prognostic systems. Patients dying within 6 months after diagnosis were correctly predicted in 60% of cases with the Lynch-index with a sensitivity of 82%. With the Najean-index 40% of these patients are correctly predicted, this index has a sensitivity of 100%. More accurate are the prognostic criteria proposed by Camitta et al [5]. With these criteria, this rapidly fatal group is correctly predicted in 85% of the patients, indicating that 15% of the patients are incorrectly predicted to have a limited survival. The sensitivity, however, is 100%. The Lohrmann-index, based on reticulocyte count predicts 64% of this group with severe aplasia. None of these prognostic systems do accurately predict long survival. We suggest that the best differentiation between patients with a long-term prognosis (more than 5 years) and patients who die from aplastic anaemia within 5 years, is made by re-evaluating the leucocyte and platelet count 3 months after the initial diagnosis. Decrease in blood counts (over 10%) predicts death from aplastic anaemia within 5 years correctly in all patients; stable or increased blood counts predict long survival in 75% of the patients.
我们评估了43例再生障碍性贫血患者中不同预后系统的准确性。使用林奇指数(Lynch-index),60%的患者在诊断后6个月内死亡的情况被正确预测,其敏感度为82%。使用纳让指数(Najean-index),这些患者中有40%被正确预测,该指数的敏感度为100%。卡米塔等人[5]提出的预后标准更为准确。依据这些标准,85%的患者中快速致命组被正确预测,这表明15%的患者被错误地预测为生存期有限。然而,其敏感度为100%。基于网织红细胞计数的洛尔曼指数(Lohrmann-index)预测了该组严重再生障碍性贫血患者中的64%。这些预后系统均无法准确预测长期生存情况。我们建议,通过在初次诊断3个月后重新评估白细胞和血小板计数,能够最好地区分长期预后(超过5年)的患者和5年内死于再生障碍性贫血的患者。血细胞计数下降(超过10%)在所有患者中均能正确预测5年内死于再生障碍性贫血的情况;血细胞计数稳定或升高在75%的患者中预测为长期生存。