Gordon-Smith E C, Gordon M Y
Ciba Found Symp. 1981;84:87-108. doi: 10.1002/9780470720660.ch6.
Aplastic anaemia can be defined as pancytopenia in the presence of a hypoplastic bone marrow and in the absence of leukaemia or malignant infiltration. Most cases have been attributed to an intrinsic defect in the pluripotential haemopoietic stem cell; however, stem cell suppression or a defect in the marrow environment would also produce the symptoms of aplasia. In vitro culture systems have been used to explore these possibilities and have provided evidence that the syndrome known as aplastic anaemia includes several distinct disease processes. These include primary failure of the haemopoietic stem cell and the suppression of stem cell function by cellular, humoral or other environmental factors. This heterogeneity provides the background for attempting to treat the disease by bone marrow transplantation, immunosuppression or stimulation of haemopoiesis.
再生障碍性贫血可定义为骨髓造血功能低下且无白血病或恶性浸润情况下的全血细胞减少。大多数病例归因于多能造血干细胞的内在缺陷；然而，干细胞抑制或骨髓环境缺陷也会产生再生障碍的症状。体外培养系统已被用于探索这些可能性，并提供了证据表明再生障碍性贫血综合征包括几种不同的疾病过程。这些包括造血干细胞的原发性衰竭以及细胞、体液或其他环境因素对干细胞功能的抑制。这种异质性为尝试通过骨髓移植、免疫抑制或刺激造血来治疗该疾病提供了背景。
