Nissen C, Moser Y, Speck B, Gratwohl A, Weis J
Br J Haematol. 1985 Nov;61(3):499-512. doi: 10.1111/j.1365-2141.1985.tb02854.x.
Serum from patients with aplastic anaemia contains two distinct stimulatory activities on haemopoiesis in culture. The first is a highly unstable enhancing activity, which mainly stimulates colony formation from BFU-E and macrophage precursors, and only acts when added directly to target bone marrow cultures. It is destroyed by Sephadex G-150 chromatography, and thus differs from colony stimulating activity (CSA) and burst promoting activity (BPA). Its mode of action is unknown. It was elevated in 70/97 patients with severe aplastic anaemia (SAA). 71 of these 97 patients were treated with high dose immunosuppression. 55/71 who achieved self-sustaining haemopoiesis had higher serum stimulating activity on BFU-E than 16/71 who never achieved remission (P = 0.0004). It was predictive of response as an all or none phenomenon, independent of the time required for recovery. It was, however, unsatisfactory as a single prognostic test. Bone marrow reconstitution also occurred in 4/71 patients whose serum inhibited BFU-E in direct culture. The second stimulator acts via enhancement of CSA- and BPA-release by accessory cells and is therefore termed 'releaser' activity. It was elevated in 27 of 51 aplastic anaemia patients and did not correlate with direct stimulatory activity. High 'releaser' activity was not predictive of response in 42 patients treated with high dose immunosuppression. However, the ability of patient cells to respond to autologous 'releaser' activity was a positive risk factor. Patients whose cells released an excess of CSA in the presence of autologous serum had a significantly higher chance of autologous recovery within 3 months than patients who produced little or no CSA in the presence of normal or excess releaser activity (P less than 0.0001). A scoring system which includes these two good risk factors is proposed for estimation of a patient's probability to recover autologous bone marrow reconstitution.
再生障碍性贫血患者的血清对培养中的造血具有两种不同的刺激活性。第一种是高度不稳定的增强活性，主要刺激BFU-E和巨噬细胞前体的集落形成，并且仅在直接添加到目标骨髓培养物中时才起作用。它可被葡聚糖G-150层析破坏，因此不同于集落刺激活性（CSA）和爆式促进活性（BPA）。其作用方式尚不清楚。在97例重型再生障碍性贫血（SAA）患者中有70例其水平升高。这97例患者中有71例接受了大剂量免疫抑制治疗。71例中实现自我维持造血的55例患者的血清对BFU-E的刺激活性高于71例中未达到缓解的16例患者（P = 0.0004）。它作为一种全或无现象可预测反应，与恢复所需时间无关。然而，作为单一的预后检测并不理想。在71例患者中有4例患者的血清在直接培养中抑制BFU-E，但骨髓也实现了重建。第二种刺激物通过增强辅助细胞释放CSA和BPA起作用，因此被称为“释放因子”活性。在51例再生障碍性贫血患者中有27例其水平升高，并且与直接刺激活性无关。在42例接受大剂量免疫抑制治疗的患者中，高“释放因子”活性不能预测反应。然而，患者细胞对自身“释放因子”活性作出反应的能力是一个阳性危险因素。在自身血清存在下细胞释放过量CSA的患者在3个月内自体恢复的机会明显高于在正常或过量释放因子活性存在下产生很少或不产生CSA的患者（P小于0.0001）。提出了一种包括这两个良好危险因素的评分系统，用于估计患者自体骨髓重建恢复的可能性。
