Abstract

GUTIERREZ, Roberto et al. SYSTEMIC THROMBOTIC MICROANGIOPATHY ASSOCIATED WITH COMPLEMENT PATHWAY MUTATIONS IN LIVING DONOR KIDNEY TRANSPLANT. CASE REPORT. Rev. nefrol. dial. traspl. [online]. 2020, vol.40, n.2, pp.139-145. ISSN 2346-8548.

Hemolytic uremic syndrome (HUS) is characterized by the presence of hemolytic anemia, thrombocytopenia and acute kidney injury. Although it is usually distinguished as typical or infectious and atypical, it is necessary to recognize clinical situations in which it is revealed, such as pregnancy, immediate postpartum period, tumors, transplantation, drugs, etc., i.e. clinical conditions that have been called complement-amplifying conditions.

Post-transplantation recurrence of atypical hemolytic uremic syndrome (aHUS) has been described in variable percentages in patients with mutations of factor H, factor B, factor I and C3, and thrombomodulin gene, in reports of isolated cases. We present the case of a patient with chronic kidney disease (CKD) secondary to renal agenesis, a preemptive recipient of a related living donor kidney, which presented renal graft dysfunction secondary to thrombotic microangiopathy, associated with neurological complications, hemorrhages, multiple organ dysfunction and death. The findings of the genetic and pathological autopsy study are described.

Keywords : thrombotic microangiopathy, TAM, atypical hemolytic uremic syndrome, aHUS; kidney transplant; trombomodulin; chronic kidney disease.

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