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- Title
Thrombotic microangiopathy following haematopoietic stem cell transplant.
- Authors
Seaby, Eleanor G.; Gilbert, Rodney D.
- Abstract
Thrombotic microangiopathy is a potentially lethal complication of haematopoietic stem cell (bone marrow) transplantation. The pathophysiology is incompletely understood, although endothelial damage appears to be central. Platelet activation, neutrophil extracellular traps and complement activation appear to play key roles. Diagnosis may be difficult and universally accepted diagnostic criteria are not available. Treatment remains controversial. In some cases, withdrawal of calcineurin inhibitors is adequate. Rituximab and defibrotide also appear to have been used successfully. In severe cases, complement inhibitors such as eculizumab may play a valuable role. Further research is required to define the pathophysiology and determine both robust diagnostic criteria and the optimal treatment.
- Subjects
ENZYME inhibitors; RITUXIMAB; BLOOD platelet activation; EPITHELIAL cells; EXTRACELLULAR space; HEMATOPOIETIC stem cell transplantation; THROMBOCYTOPENIA; DIAGNOSIS
- Publication
Pediatric Nephrology, 2018, Vol 33, Issue 9, p1489
- ISSN
0931-041X
- Publication type
Article
- DOI
10.1007/s00467-017-3803-4