Thrombotic thrombocytopenic purpura disease

Uncommon for a micro-hemolytic disease of the micro-vascular disease and thrombosis. Unknown etiology, with a small number of patients may be infected, pregnancy, collagen vascular disease, cancer, and genetic and other relevant drugs. Various causes of microvascular endothelial cell damage, resulting in collagen and basement membrane exposure caused platelets and fibrin deposition. In some patients with one or more of platelet aggregation factor (PAF), or the 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 mainly involved and capillaries microvascular obstruction caused multiple organ failure. The onset of intense, rapid progress, a small number of recurrent and can be slower. Clinical to 30 ~ 40-year-old female Most of the main performance for the bleeding, microvascular hemolytic anemia, neurological symptoms, fever and kidney damage, called TTP five of the levy. Neuropsychiatric symptoms often intermittent or volatility. Laboratory: platelet decreased significantly, moderate to severe anemia, reticulocyte increased blood platelet great film that, nucleated red blood cells and red blood cell debris, and in the bone marrow of the red giant compensatory proliferation of nuclear, normal coagulation checks. Skin, muscle, gums, and bone marrow biopsy in the capillary endothelial classes, small muscular arteries between the cortex and the 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 died within three months. Plasmapheresis and plasma infusion will enable 64% to 80% of the patients eased, PGI2 large dose intravenous infusion or a small number of cases will ease vincristine, splenectomy only short-term symptoms of some patients to improve joint anti-platelet drugs glucocorticoid plasma exchange can be used as adjuvant therapy.