Nimer S D, Leung D H, Wolin M J, Golde D W
Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, NY 10021.
Int J Hematol. 1994 Oct;60(3):185-9.
Immune suppression of hematopoiesis has been implicated in the pathogenesis of acquired aplastic anemia. Similarly, abnormalities of T cells and bone marrow stromal cells have been reported in aplastic anemia, as has abnormal cytokine production. Stem cell factor (SCF) (also known as kit ligand, mast cell growth factor and Steel factor) is an early acting hematopoietic growth factor that is produced by a variety of mesenchymal cells including bone marrow stromal cells. To determine whether abnormalities in the production of stem cell factor occur in aplastic anemia, we evaluated serum levels of SCF in 25 patients with aplastic anemia. The mean serum levels of SCF in aplastic anemia patients were significantly lower (2.7 +/- 1.1 ng/ml) than those found in a comparable population of 257 normal controls (3.3 +/- 1.1 ng/ml) (P = 0.011). The SCF level did not correlate with patient age, the duration of aplastic anemia or with white blood cell count, platelet count or hematocrit. Although there is no direct evidence that lower SCF serum levels contribute to the pancytopenia seen in this disorder, identification of underlying abnormalities that can result in the deficient production of stromally derived hematopoietic growth factors will be important.
造血的免疫抑制与获得性再生障碍性贫血的发病机制有关。同样,再生障碍性贫血患者也有T细胞和骨髓基质细胞异常的报道,细胞因子产生异常的情况也存在。干细胞因子(SCF)(也称为kit配体、肥大细胞生长因子和Steel因子)是一种早期作用的造血生长因子,由包括骨髓基质细胞在内的多种间充质细胞产生。为了确定再生障碍性贫血患者是否存在干细胞因子产生异常,我们评估了25例再生障碍性贫血患者的血清SCF水平。再生障碍性贫血患者的血清SCF平均水平(2.7±1.1 ng/ml)显著低于257名正常对照者(3.3±1.1 ng/ml)(P = 0.011)。SCF水平与患者年龄、再生障碍性贫血病程、白细胞计数、血小板计数或血细胞比容均无相关性。虽然没有直接证据表明较低的血清SCF水平导致了该疾病中的全血细胞减少,但识别可能导致基质来源的造血生长因子产生不足的潜在异常将很重要。