Systemic thrombotic microangiopathy associated with complement pathway mutations in living donor kidney transplant. Case report

  • Roberto Gutiérrez Unidad Renal, Hospital Universitario Fundación Favaloro, Buenos Aires, Argentina
  • Rita Marcela Fortunato Unidad Renal, Hospital Universitario Fundación Favaloro, Buenos Aires, Argentina
  • Carlos Vigliano Departamento de Patología, Hospital Universitario Fundación Favaloro, Buenos Aires, Argentina
  • Álvaro Espinoza Unidad Renal, Hospital Universitario Fundación Favaloro, Buenos Aires, Argentina
  • Alison Nava Unidad Renal, Hospital Universitario Fundación Favaloro, Buenos Aires, Argentina
  • Juan De Francesco Unidad Renal, Hospital Universitario Fundación Favaloro, Buenos Aires, Argentina
  • Pablo Miguel Raffaele Unidad Renal, Hospital Universitario Fundación Favaloro, Buenos Aires, Argentina
Keywords: thrombotic microangiopathy, TAM, atypical hemolytic uremic syndrome, aHUS; kidney transplant; trombomodulin; chronic kidney disease

Abstract

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 postpartal 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.

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Published
2020-09-14
How to Cite
1.
Gutiérrez R, Fortunato RM, Vigliano C, Espinoza Álvaro, Nava A, De Francesco J, Raffaele PM. Systemic thrombotic microangiopathy associated with complement pathway mutations in living donor kidney transplant. Case report. Rev Nefrol Dial Traspl. [Internet]. 2020Sep.14 [cited 2024Dec.22];40(2):139-45. Available from: http://revistarenal.org.ar/index.php/rndt/article/view/523
Issue
Vol. 40 No. 2 (2020): Abr.-Jun.
Section
Case Report

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