Linkage between the mechanisms of thrombocytopenia and thrombopoiesis.

摘要: Thrombocytopenia is defined as a status in which platelet numbers are reduced. Imbalance between the homeostatic regulation of generation and destruction 1 potential cause thrombocytopenia. In adults, 2-stage process entailing differentiation hematopoietic stem cells into mature megakaryocytes (MKs; known megakaryopoiesis) release platelets from MKs (known thrombopoiesis or biogenesis). Until recently, information about genetic defects responsible for congenital thrombocytopenia was only available few forms disease. However, investigations over past 15 years have identified mutations genes encoding >20 different proteins that these disorders, has advanced our understanding megakaryopoiesis thrombopoiesis. The underlying pathogenic mechanisms can be categorized (1) MK lineage commitment differentiation, (2) maturation, (3) defect release. Using developmental stage categories, we here update recently described discuss association systems