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Diagnosis and treatment of transplantation-associated thrombotic microangiopathy: real progress or are we still waiting?
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Journal Article
Diagnosis and treatment of transplantation-associated thrombotic microangiopathy: real progress or are we still waiting?
2007
Overview
Transplantation-associated thrombotic microangiopathy (TA-TMA) is an infrequent but devastating syndrome that occurs in allogeneic hematopoietic stem cell transplant recipients, and is associated with a variety of transplantation-related factors, including conditioning regimens, immunosuppressive agents, GVHD and opportunistic infections. Progress in managing this condition has been hampered by lack of a consensus definition and poor understanding of the pathophysiology of the disorder. Two different groups recently have proposed consensus definitions, yet they fail to distinguish the primary syndrome from the secondary causes, such as a variety of infections, medication exposure or other conditions. Increasing evidence suggests that TA-TMA is a multifactorial disorder that is distinct from thrombotic thrombocytopenic purpura (TTP), and likely represents the final common pathway of a number of endothelial cell insults. TA-TMA responds poorly to conventional treatment for TTP, including plasma exchange, but newer agents, including daclizumab and defibrotide show promise. In addition, other agents known to modify endothelial responses to injury, including statins, prostacyclin analogues, endothelin-receptor antagonists and free radical scavengers, may lead to improved outcomes for patients affected by this disorder.
Publisher
Nature Publishing Group UK,Nature Publishing Group
Subject
/ Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy
/ Biological and medical sciences
/ Bone marrow, stem cells transplantation. Graft versus host reaction
/ Complications and side effects
/ Hematologic and hematopoietic diseases
/ Hematopoietic Stem Cell Transplantation - adverse effects
/ Hemolytic-Uremic Syndrome - diagnosis
/ Hemolytic-Uremic Syndrome - etiology
/ Humans
/ Medicine
/ Platelet diseases and coagulopathies
/ Purpura, Thrombotic Thrombocytopenic - diagnosis
/ Purpura, Thrombotic Thrombocytopenic - etiology
/ Reagents
/ review
/ Statins
/ Thrombotic thrombocytopenic purpura
/ Transfusions. Complications. Transfusion reactions. Cell and gene therapy
