Thrombotic thrombocytopenic purpura disease

For an uncommon micro-hemolytic disease of the micro-vascular disease and thrombosis. Cause unknown, a small number of patients may be related to infection, pregnancy, collagen vascular disease, cancer, and genetic and other related drugs. Various causes damage microvascular endothelial cells, resulting in collagen and basement membrane exposure caused platelets and fibrin deposition. Certain patients plasma existence of one or more platelet aggregation factor (PAF), or lack of normal plasma in the PAF inhibitor, induced platelet aggregation. Some people in TTP in patients with isolated VWF giant polymer, in vitro platelet aggregation that VWF metabolic abnormalities may also be related to the incidence and TTP. Peripheral artery disease and mainly involved capillaries, microvascular obstruction caused multiple organ failure. The disease onset of intense, rapid progress, a small number can be slow and recurrent. Clinical to 30 ~ 40-year-old female common, mainly manifested as bleeding, microvascular of hemolytic anemia, neurological symptoms, fever and kidney damage, called TTP five of the levy. Neuropsychiatric symptoms often intermittent or volatility. Laboratory tests: platelets decreased significantly, to severe anemia, reticulocyte increased blood platelet great film that, nucleated red blood cells and red blood cell debris, and bone marrow megakaryocyte erythroid of compensatory hyperplasia, normal coagulation checks. The skin, muscle, gums and bone marrow biopsy in capillary endothelial classes, small muscular arteries within the cortex and between glass-like deposits, with vascular endothelial proliferation and luminal obstruction. Except for a small number of chronic-disease sustainable few months to a few years, 80% of patients in three months killed. Plasma exchange and plasma infusion will enable 64% to 80% of patients eased, large dose PGI2 infusion or intravenous vincristine could ease a few cases, splenectomy only to improve the symptoms of some patients short period of time, the joint anti-platelet drugs glucocorticoid the plasma exchange can be used as adjuvant therapy.