Mir M A, Geary C G
Postgrad Med J. 1980 May;56(655):322-9. doi: 10.1136/pgmj.56.655.322.
The authors summarize their experience with 174 patients with aplastic anaemia (AA) with particular reference to course, prognostic factors, conversion to other blood disorders, treatment and outcome. Aplastic anaemia was defined as pancytopenia and a hypocellular marrow at some time during the illness. Seven patients terminated with acute leukaemia, 8 developed haematological features of sideroblastic anaemia and 5 showed a red cell membrane defect commonly found in paroxysmal noctural haemoglobinuria. Complete recovery occurred in only 4 patients; 70 others showed a partial remission with some residual haematological abnormality in peripheral blood. Eighteen of these had spontaneous remission. Remission was brief in 27 patients who died; only 32 patients remained in partial remission. Twenty-five per cent. of the patients with AA run an acute course and die within 6 months of the time of diagnosis. The remainder run a subacute or chronic course, punctuated in some cases by a transient remission, but in others by a conversion to other related haematological disorders. An acute course is suggested by a rapid onset of symptoms, a falling neutrophil count, a haemoglobin level less than 5 g/dl and a very low platelet count. A chronic course is likely in those patients who have a slow onset of symptoms, a stable neutrophil count and a Hb level in excess of 5 g/dl. The authors' experience shows that the disease runs either of the 2 courses irrespective of the supportive therapy.
作者总结了他们对174例再生障碍性贫血(AA)患者的经验,特别提及病程、预后因素、转化为其他血液疾病、治疗及结果。再生障碍性贫血被定义为在疾病过程中的某个时间出现全血细胞减少和骨髓细胞减少。7例患者最终发展为急性白血病,8例出现铁粒幼细胞贫血的血液学特征,5例表现出阵发性睡眠性血红蛋白尿常见的红细胞膜缺陷。仅4例患者完全康复;其他70例患者部分缓解,外周血仍有一些残留的血液学异常。其中18例为自发缓解。27例死亡患者缓解期短暂;仅32例患者仍处于部分缓解状态。25%的再生障碍性贫血患者病程呈急性,在诊断后6个月内死亡。其余患者病程呈亚急性或慢性,有些病例有短暂缓解,而另一些则转化为其他相关血液疾病。症状迅速出现、中性粒细胞计数下降、血红蛋白水平低于5g/dl以及血小板计数极低提示病程呈急性。症状出现缓慢、中性粒细胞计数稳定且血红蛋白水平超过5g/dl的患者病程可能呈慢性。作者的经验表明,无论支持治疗如何,该疾病都呈这两种病程之一。