ERYTHROGENESIS IMPERFECTA
ERYTHROGENESIS IMPERFECTA
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DOI：
10.1136/adc.25.124.313
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发表时间：
1950-01-01
影响因子：
5.2
通讯作者：
CATHIE, IAB
中科院分区：
文献类型：
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作者：
CATHIE, IAB
骨髓中红细胞前体的正常功能对于维持足够数量的循环红细胞至关重要，并且有几种公认的方式可能会扰乱红细胞的正常产生。在某些情况下，紊乱的机制已被清楚了解，但在其他情况下，其病因却相当未知。包含再生障碍性和发育不全的各种术语通常用于定义儿童贫血，这些术语不仅经常混淆，而且有时它们应用于外周血，有时应用于骨髓状态，因此对贫血的特定描述可能与工作中的病理过程完全不一致。再生障碍性贫血通常涉及所有骨髓成分，已被报道约 150 次（Whitby 和 Britton，1946）。然而，在对所谓的再生障碍性贫血进行分类时，Rhoads 和 Miller (1934) 除了描述真正的再生障碍性骨髓外，还认识到一种骨髓“充满”可能无法成熟的原始品种前体的类型。骨髓衰竭引起的再生障碍有时被视为长期成熟因子缺乏或溶血过程的结果。 Whitby 和 Britton 在文献中提到了骨髓成红细胞成分完全再生障碍的六例，最近描述了类似但周期性的骨髓成红细胞缺乏，其中过敏因素似乎发挥了作用（Gasser，1949）。有时会出现与再生障碍性贫血的血象非常相似的外周全血细胞减少症，这被认为是由于脾脏产生抑制骨髓的物质所致。同样，一些毒物、感染和药物似乎对一种或另一种骨髓元素有直接抑制作用，导致功能障碍、发育不全或发育不全，从而导致外周缺陷。
A proper functioning of the red cell precursors in the bone marrow is essential for the maintenance of an adequate number of circulating red blood corpuscles, and there are several well-recognized ways in which the normal production oferythrocytes may be upset. The mechanism of the derangement is clearly understood in some cases, but in others the aetiology is quite unknown. Various terms containing the words aplastic and hypoplastic are in commonuse to define the anaemias of childhood, and not only are these words often confused but sometimes they are applied to the peripheral blood and sometimes to the state of the marrow, so that a given description of an anaemia may be entirely at variance with the pathological process at work. Aplastic anaemia, usually involving all the marrow elements, has been reported some 150 times (Whitby and Britton, 1946). In classifying the so-called aplastic anaemias, however, Rhoads and Miller (1934), besides describing the truly aplastic marrow, recognized a type in which the marrow was' full'of precursors of a primitive variety presumably incapable of maturing. An aplasia due to marrow exhaustion is occasionally seen as the result of a prolonged maturation factor deficiency or a haemolytic process. Whitby and Britton refer to six cases in the literature of total aplasia of the marrow erythroblastic elements, and a similar but periodic lack of marrow erythroblasts has been recently described, in which an alergic factor appears to play a part (Gasser, 1949). A peripheral pancytopenia rather similar to the blood picture in aplastic anaemia is sometimes seen, and is thought to be due to the production by the spleen of a substance inhibitory to the bone marrow. Again, some poisons, infections, and drugs appear to have a directdepressant action on one or other of the bone marrow elements, causing a dysfunction, hypoplasia, or aplasia with consequent peripheral deficiency.