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Complenentopathies

Complenentopathies are rare diseases in which the pathophysiology and organs associated damage is driven by failure to regulate compliment on cell surfaces..

Germline orsomatic variants in genes that regulate complement are frequently found, and thrombosis poorly responsive to anticoagulation is common.

Thrombosis and other end organ damage of stroke and renal failure are often triggered by complement amplifying conditions of infection, autoimmunity, surgery, pregnancy, and cancer.

Examples of complementopathies include: atypical hemolytic uremic syndrome, HELLP syndrome, catastrophic antiphospholipid antibody syndrome, and cold agglutinin disease.

 

 

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