Recurrent and de novo HUS after renal transplantation
- Christina L Klein, MD
Christina L Klein, MD
- Transplant Nephrologist
- Piedmont Transplant Institute
- Anuja Java, MD
Anuja Java, MD
- Instructor in Medicine
- Washington University School of Medicine
- Daniel C Brennan, MD, FACP
Daniel C Brennan, MD, FACP
- Editor-in-Chief — Nephrology
- Section Editor — Renal Transplantation
- Professor of Medicine
- Johns Hopkins Medical School
- Medical Director and Co-Director of The Comprehensive Transplant Center
- Department of Internal Medicine
- Division of Nephrology
Hemolytic uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia, and renal impairment. There are multiple etiologies for HUS. (See "Approach to the patient with suspected TTP, HUS, or other thrombotic microangiopathy (TMA)".)
HUS may result in end-stage renal disease (ESRD), requiring either dialytic therapy or transplantation. Among patients with ESRD due to HUS who undergo transplantation, the risk of recurrence depends upon the underlying etiology. Less commonly, patients who undergo renal transplantation for other causes of ESRD may also develop HUS.
This topic reviews recurrent and de novo HUS in renal transplant recipients. The causes, diagnosis, and treatment of HUS in nontransplant patients are discussed elsewhere. (See "Pathophysiology of acquired TTP and other primary thrombotic microangiopathies (TMAs)" and "Drug-induced thrombotic microangiopathy" and "Approach to the patient with suspected TTP, HUS, or other thrombotic microangiopathy (TMA)" and "Complement-mediated hemolytic uremic syndrome".)
Most cases of HUS in the nontransplant population, particularly among children, are secondary to infection with Shiga toxin-producing Escherichia coli serotypes and Streptococcus pneumoniae. Other causes include human immunodeficiency virus (HIV), pregnancy, cancer, and medications (eg, quinine, chemotherapeutic agents, and immunosuppressive drugs such as calcineurin inhibitors and mammalian [mechanistic] target of rapamycin [mTOR] inhibitors) (table 1). HUS may also be caused by genetic defects in complement proteins that regulate the alternative pathway, leading to dysregulated complement activation. This is referred to as "complement-mediated HUS" or "atypical HUS." The mutations may be familial or sporadic. (See "Complement-mediated hemolytic uremic syndrome".)
HUS that occurs in the transplant recipient may be recurrent or de novo. Nearly all patients with recurrent HUS have a complement-mediated disease that produced both the pretransplant HUS and the recurrence after transplantation . De novo HUS may be due to any of the etiologies that cause thrombotic microangiopathy in the general population or may be related to the transplantation. Causes of thrombotic microangiopathy that are common among transplant recipients include immunosuppressive drugs [2,3], ischemia reperfusion injury , viral infections, and antibody-mediated rejection [5-8]. (See 'De novo HUS' below and "Drug-induced thrombotic microangiopathy".)
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- Recurrent HUS
- De novo HUS
- CLINICAL PRESENTATION
- PREVENTION OF RECURRENT HUS
- Screening for mutations
- Selection of donor kidney
- Prophylactic therapy
- Patients with recurrent HUS
- Patients with de novo HUS
- SUMMARY AND RECOMMENDATIONS