Recurrent and de novo HUS or TTP after renal transplantation
- Christina L Klein, MD
Christina L Klein, MD
- Transplant Nephrologist
- Piedmont Transplant Institute
- Daniel C Brennan, MD, FACP
Daniel C Brennan, MD, FACP
- Editor-in-Chief — Nephrology
- Section Editor — Renal Transplantation
- Professor of Medicine
- Washington University School of Medicine
Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are characterized by hemolytic anemia, thrombocytopenia, and renal impairment. There are multiple different etiologies for TTP and HUS. (See "Approach to the patient with suspected TTP, HUS, or other thrombotic microangiopathy (TMA)".)
TTP or HUS may result in end-stage renal disease (ESRD), requiring either dialytic therapy or transplantation. Among patients with ESRD due to TTP or 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 TTP or HUS.
This topic reviews recurrent and de novo TTP and HUS in renal transplant recipients. The causes, diagnosis, and treatment of TTP and HUS in non-transplant patient are discussed elsewhere. (See "Pathophysiology of acquired TTP and other primary thrombotic microangiopathies (TMAs)" and "Acquired TTP: Clinical manifestations and diagnosis" and "Acquired TTP: Initial treatment" and "Drug-induced thrombotic microangiopathy" and "Approach to the patient with suspected TTP, HUS, or other thrombotic microangiopathy (TMA)" and "Hereditary thrombotic thrombocytopenic purpura (TTP)" and "Complement-mediated hemolytic uremic syndrome".)
Most cases of TTP or HUS in the non-transplant 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, antiplatelet agents ticlopidine and clopidogrel, chemotherapeutic agents, and immunosuppressive drugs used in organ transplantation such as calcineurin inhibitors and mammalian (mechanistic) target of rapamycin [mTOR] inhibitors) (table 1). Rarely, HUS is caused by genetic defects in complement proteins that regulate the alternative pathway, leading to dysregulated complement activation. These mutations may be familial or sporadic. (See "Complement-mediated hemolytic uremic syndrome".)
TTP or HUS that occurs in the transplant recipient may be recurrent or de novo. De novo TTP or HUS may have any of the underlying etiologies, as in the general population, or be related to the transplantation. Causes of thrombotic microangiopathy that are common among transplant recipients include immunosuppressive drugs [1,2], ischemia reperfusion injury , and viral infections [4-7]. (See 'Epidemiology' below and "Drug-induced thrombotic microangiopathy".)
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