Fohlmeister I, Fischer R, Mödder B, Rister M, Schaefer H E
J Clin Pathol. 1985 Nov;38(11):1218-24. doi: 10.1136/jcp.38.11.1218.
In a retrospective study of 111 patients with aplastic anaemia iliac crest biopsies were evaluated for the presence of morphological features statistically related to the evolution of the disease. Prognostic variables for a transition to acute non-lymphatic leukaemia were: cellular atypias of the three haemopoietic lineages, as observed in the myelodysplastic syndrome, and especially "micromegakaryocytes"; high numbers or irregular distribution of megakaryocytes, or both; and (slight) marrow fibrosis. Clinical variables did not influence these prognostic correlations. Prognosis in relation to death from bone marrow failure without leukaemia might well have been influenced by a strong plasma cell reaction, but this correlation was weakened by clinical factors. On the basis of this study aplastic anaemia can thus be subdivided morphologically into two disease entities--namely, hypocellular myelodysplastic syndrome with a 23-82% risk of acute non-lymphatic leukaemia developing within three years, depending on how many variables associated with acute non-lymphatic leukaemia are present, and non-dysplastic myelohypoplasia.
在一项对111例再生障碍性贫血患者的回顾性研究中,对髂嵴活检进行评估,以确定是否存在与疾病进展有统计学关联的形态学特征。向急性非淋巴细胞白血病转变的预后变量包括:在骨髓增生异常综合征中观察到的三种造血谱系的细胞异型性,尤其是“微巨核细胞”;巨核细胞数量多或分布不规则,或两者皆有;以及(轻度)骨髓纤维化。临床变量不影响这些预后相关性。与无白血病的骨髓衰竭死亡相关的预后很可能受到强烈浆细胞反应的影响,但这种相关性因临床因素而减弱。基于这项研究,再生障碍性贫血因此可在形态学上分为两种疾病实体——即低细胞性骨髓增生异常综合征,根据存在与急性非淋巴细胞白血病相关变量的数量,三年内发生急性非淋巴细胞白血病的风险为23%至82%,以及非发育异常性骨髓造血功能减退。