Abstract: FR-PO756
Non-ADAMTS13-Mediated Hemolytic Uremic Syndrome (HUS) in a Kidney Transplant Recipient due to SARS-CoV-2 Infection and Its Response to PLEX: A Case Report
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
- Momand, David A., Loma Linda University Medical Center, Loma Linda, California, United States
- Regmi, Surakshya, Loma Linda University Medical Center, Loma Linda, California, United States
- Ganesan, Lakshmi, Loma Linda University Medical Center, Loma Linda, California, United States
- Villicana, Rafael, Loma Linda University Medical Center, Loma Linda, California, United States
- Raza, Aamir, Loma Linda University Medical Center, Loma Linda, California, United States
- Akram, Sami M., Loma Linda University Medical Center, Loma Linda, California, United States
Introduction
During the COVID-19 pandemic many new complications in kidney transplant recipients have arisen. One such entity is Hemolytic Uremic Syndrome characterized by Thrombotic Microangiopathy. Here, we report a rare case of non-ADAMTS-13 and non-complement mediated TMA in a kidney transplant recipient with mild COVID-19 disease.
Case Description
27 y/o F with a history of GN-mediated ESRD, status post DDKTx in April 2016, thymo induction, on tacrolimus, mycophenolate mofetil and prednisone, presented with a two-day history of fevers and diffuse myalgias. Lab work-up positive for COVID19 and AKI with Cr 4.6 mg/dL and oliguria (baseline Cr 0.8 mg/dL). On day 3, platelets decreased to 68K cells/mm3. Peripheral smear performed showing many schistocytes with low haptoglobin and elevated LDH. C3 and C4 were normal. Alternate complement pathway function (Mayo laboratory) was normal. Negative for Shiga toxin in stool and the ADAMTS13 activity 73%. Von Willebrand Factor distribution normal. PLEX initiated with high dose steroids with resolution of schistocyte index and AKI.
Discussion
HUS after COVID19 has been reported. The virus can cause HUS directly or indirectly by reducing ADAMTS13. The distribution of vWF multimers can be altered by the virus. A case of Dengue virus causing non-ADAMTS13 HUS has been reported before. Another mechanism of HUS/TMA is dysfunction of alternative complement pathway (inherited or acquired). Also, complement inhibitors such as Eculizumab may not be helpful. Empiric therapy with PLEX was helpful as illustrated by schistocyte response. To the best of our knowledge this is the first case report showing a schistocyte response with two sessions of PLEX in a SARS-CoV-2 mediated HUS.
| Differential Diagnosis | Typical HUS | Complement-mediated atypical HUS | Atypical HUS due to COVID19 | TTP |
| Background | No gastroenteritis | No prior C3GN/aHUS | Recent SARS-CoV-2 infection | No history |
| Testing/Diagnosis | Shiga toxin negative | Complements normal. Alternate complement function S test normal. | UNKNOWN MECHANISM | ADAMTS13 is 73% (normal). vWF distribution normal |
| Pathophysiology | Shiga toxin mediated endothelial injury | Alternate complement pathway dysregulation (congenital or acquired) | Non-complement mediated endothelial injury | ADAMTS13 deficiency |
| Therapy | Supportive care | PLEX + Eculizumab | PLEX + Steroids | PLEX + Steroids |