Özet:

Atypic hemolytic urethral syndrome is a rare form of thrombetic microangiopathy that occurs from chronic uncontrolled activation of the path of the alternative compliment. Atypic hemolithic urethral syndrome is associated with non-immune hemolithic anemia, thrombocytopenia and kidney constipation. The disease can cause mortality and morbidity in the acute period and long-term renal failure in the last period. Despite the number of kidney stagnations in the disease, extra-renal stagnations can be seen in 20% of cases. In the diagnosed patients, genetic study should be carried out in order to preserve kidney functions and ensure hematological remission. In the first 24 hours after the diagnosis, the first stage of treatment should be initiated with fresh frozen plasma, and, if necessary, should be transferred to eculuzimab treatment. In recent period of renal insufficiency in advanced cases, the liver or liver transplant can be planned along with eculzimab treatment.

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