Thrombotic microangiopathies assessment: mind the complement
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 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...
متن کاملUpdate on the role of the complement system in the pathogenesis of thrombotic microangiopathies.
In thrombotic microangiopathies (TMA) pathological changes of the small vessels are present, which lead to ischaemia of the affected tissues, low platelet-count and intravascular haemolytic anaemia with fragmentocytes. Two main clinical syndromes belong to the group of TMAs: the haemolytic uraemic syndrome (HUS) with kidney failure, mainly affecting children, and the thrombotic thrombocytopenic...
متن کاملComplement C5-inhibiting therapy for the thrombotic microangiopathies: accumulating evidence, but not a panacea
Thrombotic microangiopathy (TMA), characterized by organ injury occurring consequent to severe endothelial damage, can manifest in a diverse range of diseases. In complement-mediated atypical haemolytic uraemic syndrome (aHUS) a primary defect in complement, such as a mutation or autoantibody leading to over activation of the alternative pathway, predisposes to the development of disease, usual...
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ژورنال
عنوان ژورنال: Clinical Kidney Journal
سال: 2020
ISSN: 2048-8513
DOI: 10.1093/ckj/sfaa195