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Recurrent and de novo TTP-HUS after renal transplantation

Christina L Klein, MD
Daniel C Brennan, MD, FACP
Section Editor
Barbara Murphy, MB, BAO, BCh, FRCPI
Deputy Editor
Alice M Sheridan, MD


Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is characterized by hemolytic anemia, thrombocytopenia, and renal impairment. There are multiple different etiologies for TTP-HUS. (See "Pathophysiology of acquired TTP and other primary thrombotic microangiopathies (TMAs)".)

TTP-HUS may result in end-stage renal disease (ESRD), requiring either dialytic therapy or transplantation. Among patients with ESRD due to TTP-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-HUS; this is termed de novo TTP-HUS.

This topic reviews recurrent and de novo TTP-HUS in renal transplant recipients. The causes, diagnosis, and treatment of TTP-HUS in the 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)".)


Most cases of TTP-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 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-HUS that occurs in the transplant recipient may be recurrent or de novo. De novo TTP-HUS may have any of the underlying etiologies, like TTP-HUS in the general population, or be related to the transplantation. Causes of TTP-HUS that are common among transplant recipients include immunosuppressive drugs [1,2], ischemia reperfusion injury [3], and viral infections [4-7]. (See 'Epidemiology' below.)


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Literature review current through: Jan 2016. | This topic last updated: Jan 13, 2016.
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