Complement Activation and Thrombotic Microangiopathies
نویسندگان
چکیده
منابع مشابه
Thrombotic Microangiopathies
Thrombotic microangiopathy results from thrombotic occlusion of the microvasculature leading to fragmentation of red blood cells, profound thrombocytopenia, and a microangiopathic hemolytic anemia with elevation of lactate dehydrogenase and negative direct Coomb's test. This constellation of clinical and laboratory findings is not due to one disease entity; rather, it represents a variety of un...
متن کاملExtra-Renal Manifestations of Complement-Mediated Thrombotic Microangiopathies
Thrombotic microangiopathies (TMA) are rare but severe disorders, characterized by endothelial cell activation and thrombus formation leading to hemolytic anemia, thrombocytopenia, and organ failure. Complement over activation in combination with defects in its regulation is described in an increasing number of TMA and if primary for the disease denominated as atypical hemolytic-uremic syndrome...
متن کاملComplement activation: the missing link between ADAMTS-13 deficiency and microvascular thrombosis of thrombotic microangiopathies.
Endothelial injury is the central factor in the events leading to thrombotic microangiopathy (TMA); however, the mechanisms involved are not fully understood. Here we investigate the role of neutrophils (PMNs) and of complement activation in inducing microvascular damage and loss of thromboresistance in TMA associated with ADAMTS-13 deficiency. PMNs isolated during the acute phase of the diseas...
متن کاملComplement activation in thrombotic microangiopathy.
The endothelium lining the vascular lumen is continuously exposed to complement from the circulation. When erroneously activated on host cells, complement may generate a deleterious effect on the vascular wall leading to endothelial injury, exposure of the subendothelial matrix and platelet activation. In this review the contribution of complement activation to formation and maintenance of the ...
متن کاملEndothelial Pathology in Thrombotic Microangiopathies
Introduction Thrombotic microangiopathy is a term for a morphologic lesion characterized by platelet and fibrin thrombi involving the microvasculature. The morphologic lesions thus overlap between hemolytic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), and the lesions seen in scleroderma and malignant hypertension. HUS/TTP typically involves glomeruli and smaller vessels, whereas s...
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ژورنال
عنوان ژورنال: Clinical Journal of the American Society of Nephrology
سال: 2019
ISSN: 1555-9041,1555-905X
DOI: 10.2215/cjn.05830519