Abstract: FR-PO757
Post-Transplant Thrombotic Microangiopathy as a Manifestation of Atypical Haemolytic Uraemic Syndrome of Primary Origin
Session Information
- Post-Transplantation and Case Reports
November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Transplantation
- 2102 Transplantation: Clinical
Authors
- Aguilar, Rita Belinda, Hospital Universitario Dr Jose Eleuterio Gonzalez, Monterrey, Nuevo León, Mexico
- Flores Mendoza, Allina Primavera, Hospital Universitario Dr Jose Eleuterio Gonzalez, Monterrey, Nuevo León, Mexico
- Guerrero Gonzalez, Elisa Maria, Hospital Universitario Dr Jose Eleuterio Gonzalez, Monterrey, Nuevo León, Mexico
- Olivo Gutierrez, Mara Cecilia, Hospital Universitario Dr Jose Eleuterio Gonzalez, Monterrey, Nuevo León, Mexico
- Gomez Villarreal, Juan Pablo, Hospital Universitario Dr Jose Eleuterio Gonzalez, Monterrey, Nuevo León, Mexico
- Rizo Topete, Lilia Maria, Hospital Universitario Dr Jose Eleuterio Gonzalez, Monterrey, Nuevo León, Mexico
Introduction
Several conditions could trigger endothelial injury that leads to Thrombotic Microangiopathy (MAT) which can have primary and secondary etiology. If transplant associated, early diagnosis and aggressive treatment should be done in order to preserve allograft function.
Case Description
42 year old woman with history of CKD and kidney allograft rejection eighteen years ago. She underwent second kidney transplant from deceased donor, induction immunosuppression included methylprednisolone and thymoglobulin. On day five post-op she developed abrupt onset anuria, delayed graft function and laboratory showed anemia, thrombocytopenia elevated LDH, schistocytes on peripheral smear and normal ADAMST-13 activity, autoimmune screen and coagulation parameters. She also developed surgery associated complications that derived in surgical reintervention. Kidney allograft biopsy was performed and it showed swollen endothelial cells, fibrin-platelet thrombi within glomerular capillaries and negative C4d stain (Fig 1), genetic testing revealed a variant on DGKe gene. Therapeutic plasma exchange was done, soon after patient kidney function improved and started treatment with anti-C5 monoclonal antibody, renal function is successful and stable.
Discussion
In this case we faced a patient who developed what it appears to be an Atypical Haemolytic Uraemic Syndrome (aHUS). Ischemia-reperfusion injury and antibody mediated rejection, secondary causes as well as infections were ruled out. Based on genetic testing aHUS is presumed to be of primary origin.