title: Post-bone marrow transplant thrombotic microangiopathy.
abstract: Thrombotic microangiopathy (TMA) is a systemic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia and organ failure. Post-bone marrow transplant TMA (post-BMT TMA) is a life-threatening condition that has been reported to afflict between 0.5 and 63.6% of BMT patients. The incidence of post-BMT TMA is affected by evolving therapies such as conditioning regimens. The etiology of post-BMT TMA is thought to be multifactorial, including the effects of immunosuppressive agents, viral infections, TBI and GvHD. A growing body of evidence highlights the importance of complement system activation and endothelial damage in post-BMT TMA. Although plasmapheresis has commonly been used, its therapeutic rationale for the majority of post-BMT TMA cases is unclear in the absence of circulatory inhibitors. It has become possible to target complement activation with eculizumab, a drug that blocks the terminal complement pathway. Early studies have highlighted the importance of anti-complement therapies in treating post-BMT TMA. Moreover, finding complement gene mutations may identify patients at risk, but whether such patients benefit from prophylactic anti-complement therapies before BMT remains to be studied. This review focuses on diagnostic criteria, pathophysiology, treatment and renal outcomes of post-BMT TMA.
authors.affiliation: Transplantation Research Center, Department of Medicine, Renal Division, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: F
authors.initials: F
authors.lastname: Obut
authors.affiliation: Transplantation Research Center, Department of Medicine, Renal Division, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: V
authors.initials: V
authors.lastname: Kasinath
authors.affiliation: Transplantation Research Center, Department of Medicine, Renal Division, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: R
authors.initials: R
authors.lastname: Abdi
mesh_terms: D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D003167:Complement Activation / Q000187:drug effects / Q000235:genetics; D004730:Endothelium, Vascular / Q000473:pathology; D006801:Humans; D007674:Kidney Diseases / Q000209:etiology; D011292:Premedication / Q000379:methods; D057049:Thrombotic Microangiopathies / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy*