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Emerging Concepts in Hematopoietic Stem Cell Transplantation–Associated Renal Thrombotic Microangiopathy and Prospects for New Treatments

  • Rimda Wanchoo
    Correspondence
    Address for Correspondence: Rimda Wanchoo, MD, Division of Kidney Diseases and Hypertension, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, 100 Community Dr, Great Neck, NY 11021
    Affiliations
    Division of Kidney Diseases and Hypertension, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY
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  • Ruthee L. Bayer
    Affiliations
    Division of Hematology and Oncology and the Northwell Cancer Institute, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY
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  • Claude Bassil
    Affiliations
    Division of Nephrology and Hypertension, University of South Florida, Tampa, FL

    Renal Service, H. Lee Moffitt Center, Tampa, FL
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  • Kenar D. Jhaveri
    Affiliations
    Division of Kidney Diseases and Hypertension, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY
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Published:August 23, 2018DOI:https://doi.org/10.1053/j.ajkd.2018.06.013
      Thrombotic microangiopathy associated with hematopoietic stem cell transplantation (HSCT-TMA) is a well-recognized complication of HSCT that has a high risk for death. Even in patients who survive, HSCT-TMA is associated with long-term morbidity and chronic organ injury. HSCT-TMA is a multisystem disease that often affects the kidneys. Renal manifestations of HSCT-TMA include reduced glomerular filtration rate, proteinuria, and hypertension. Understanding of the pathophysiology of HSCT-TMA has expanded in the last decade. Endothelial injury plays a major role. Recent studies also suggest involvement of complement activation. HSCT-TMA has also been considered by some to be an endothelial variant of graft-versus-host disease. Understanding the pathophysiology of HSCT-TMA and its association with activation of the complement system may aid in developing novel therapeutic options. In this review, we summarize current knowledge focusing on epidemiology and prognosis, evidence of complement activation, and endothelial injury; the possible link to graft-versus-host disease; and treatment options for HSCT-TMA.

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