Islam A
Department of Medical Oncology, Roswell Park Memorial Institute, Buffalo, New York 14263.
Med Hypotheses. 1988 Apr;25(4):209-17. doi: 10.1016/0306-9877(88)90032-1.
Idiopathic aplastic anaemia (AA), aplastic anaemia of unknown aetiology, is usually defined as marrow failure with fatty replacement of hemopoietic tissue and peripheral pancytopenia. The pathophysiology is largely unknown, though many mechanisms have been hypothesized. These include the absence of or defects in hemopoietic stem cells (HSC), abnormalities of the bone marrow (BM) microenvironment, immune system disorders and abnormalities of the regulatory factors that control hemopoiesis. The characteristic feature of AA is the replacement of hematopoietically active marrow by fat cells; however, the fat cells themselves have received little attention to date, and this apparent fatty marrow infiltration has been considered a secondary phenomenon. That the marrow fat cells in AA may be abnormal and may have a pathogenic role has never been considered. This communication, postulates that AA may result from an abnormal and excessive proliferation of marrow fat cells and the displacement of the hematopoietic tissue of the marrow; and that the resultant marrow failure could be a secondary phenomenon.
特发性再生障碍性贫血（AA），即病因不明的再生障碍性贫血，通常被定义为骨髓衰竭伴造血组织脂肪替代和外周全血细胞减少。尽管已经提出了许多机制，但病理生理学在很大程度上仍不清楚。这些机制包括造血干细胞（HSC）的缺失或缺陷、骨髓（BM）微环境异常、免疫系统紊乱以及控制造血的调节因子异常。AA的特征性表现是造血活跃的骨髓被脂肪细胞替代；然而，脂肪细胞本身迄今为止很少受到关注，这种明显的脂肪骨髓浸润一直被认为是一种继发性现象。AA中的骨髓脂肪细胞可能异常并可能具有致病作用这一点从未被考虑过。本通讯推测，AA可能是由于骨髓脂肪细胞异常过度增殖以及骨髓造血组织被取代所致；并且由此导致的骨髓衰竭可能是一种继发性现象。
