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Thrombotic microangiopathy following haematopoietic stem cell transplant

Thrombotic microangiopathy following haematopoietic stem cell transplant
Thrombotic microangiopathy following haematopoietic stem cell transplant

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.

Acute kidney injury, Complement, Hematopoietic stem cell transplant, Neutrophil extracellular traps, Thrombotic microangiopathy
0931-041X
1489-1500
Seaby, Eleanor G.
ec948f42-007c-4bd8-9dff-bb86278bf03f
Gilbert, Rodney D.
c69c5b99-2a15-4502-acd8-36b254d42601
Seaby, Eleanor G.
ec948f42-007c-4bd8-9dff-bb86278bf03f
Gilbert, Rodney D.
c69c5b99-2a15-4502-acd8-36b254d42601

Seaby, Eleanor G. and Gilbert, Rodney D. (2018) Thrombotic microangiopathy following haematopoietic stem cell transplant. Pediatric Nephrology, 33 (9), 1489-1500. (doi:10.1007/s00467-017-3803-4).

Record type: Article

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.

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Accepted/In Press date: 1 September 2017
e-pub ahead of print date: 9 October 2017
Published date: 1 September 2018
Keywords: Acute kidney injury, Complement, Hematopoietic stem cell transplant, Neutrophil extracellular traps, Thrombotic microangiopathy

Identifiers

Local EPrints ID: 422782
URI: http://eprints.soton.ac.uk/id/eprint/422782
ISSN: 0931-041X
PURE UUID: fd950d60-557d-4f3c-854f-cd11b03420a1
ORCID for Eleanor G. Seaby: ORCID iD orcid.org/0000-0002-6814-8648
ORCID for Rodney D. Gilbert: ORCID iD orcid.org/0000-0001-7426-0188

Catalogue record

Date deposited: 06 Aug 2018 16:30
Last modified: 18 Apr 2024 01:54

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Contributors

Author: Eleanor G. Seaby ORCID iD
Author: Rodney D. Gilbert ORCID iD

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