Strauss G, Ballmaier M, Schulze H, Bogenberger J, Riehm H, Welte K
Abteilung Pädiatrische Hämatologie und Onkologie, Medizinische Hochschule Hannover.
Klin Padiatr. 1996 Jul-Aug;208(4):168-71. doi: 10.1055/s-2008-1046468.
Thrombopoietin (TPO) belongs to the family of hematopoietic growth factors. It supports the growth and differentiation of megakaryocytic progenitors and precursors in vitro and in vivo. The predominant site of production of TPO is the liver. However, regulation of TPO serum levels seems to be not due to transcriptional regulation in the liver but due to degradation of circulating TPO by platelets. Thrombopoietin serum levels in children with thrombocytopenia associated with aplastic anemias, Fanconi anemia and TAR syndrome are elevated whereas in children with idiopathic thrombocytopenia the TPO levels are normal. The defective activation of platelets by TPO in a children with TAR syndrome suggests a defective response of megakaryocytopoiesis to TPO.
血小板生成素（TPO）属于造血生长因子家族。它在体外和体内均支持巨核细胞祖细胞和前体细胞的生长与分化。TPO的主要产生部位是肝脏。然而，TPO血清水平的调节似乎并非由于肝脏中的转录调节，而是由于血小板对循环中TPO的降解。再生障碍性贫血、范可尼贫血和血小板减少伴桡骨缺失综合征（TAR综合征）患儿的血小板生成素血清水平升高，而特发性血小板减少症患儿的TPO水平正常。TAR综合征患儿中TPO对血小板的激活缺陷提示巨核细胞生成对TPO的反应存在缺陷。
