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伴有“热区”的再生障碍性贫血

Aplastic anaemia with 'hot pockets'.

作者信息

Kansu E, Erslev A J

出版信息

Scand J Haematol. 1976 Nov;17(5):326-34.

PMID:1034334
Abstract

4 patients with chronic severe aplastic anaemia and with persistent foci of intense haematopoietic activity, so-called 'Hot Pockets', in their bone marrow were studied. In all patients the remainder of the bone marrow was morphologically and erythrokinetically hypoplastic. The cellular morphology in these 'Hot Pockets' displayed megaloblastic features and definite maturation abnormalities in erythroblasts, but no consistent changes in the myeloid or megakaryocytic series. The presence of persistent 'Hot Pockets' presents a conceptual challenge since these pockets contain multipotential stem cells capable of differentiation and self-renewal, but obviously incapable of repopulation of the bone marrow. In view of additional evidence for dyserythropoiesis in aplastic anaemia such as changes in erythroblast morphology and the production of macrocytes, fetal haemoglobin and complement sensitive cells, it seems likely that the bone marrow of aplastic anaemia patients is totally dyserythropoietic rather than hypoactive and that bone marrow transplantation in many cases may be both justified and necessary.

摘要

对4例慢性重型再生障碍性贫血患者进行了研究,这些患者骨髓中存在所谓的“热点区”,即持续存在的强烈造血活动灶。所有患者骨髓的其余部分在形态学和红细胞动力学方面均发育不全。这些“热点区”的细胞形态表现为巨幼样特征,成红细胞存在明确的成熟异常,但髓系或巨核细胞系未见一致变化。持续存在的“热点区”的出现带来了一个概念上的挑战,因为这些区域含有能够分化和自我更新的多能干细胞,但显然无法重新填充骨髓。鉴于再生障碍性贫血中红细胞生成异常的其他证据,如成红细胞形态的改变以及大红细胞、胎儿血红蛋白和补体敏感细胞的产生,再生障碍性贫血患者的骨髓似乎完全存在红细胞生成异常而非活性低下,并且在许多情况下骨髓移植可能是合理且必要的。

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