Diagnostic and risk criteria for HSCT-associated thrombotic microangiopathy: a study in children and young adults

Transplant-associated thrombotic microangiopathy (TMA) leads to generalized endothelial dysfunction that can progress to multiorgan injury, and severe cases are associated with poor outcomes after hematopoietic stem cell transplantation (HSCT). Identifying patients at highest risk for severe disease...

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Bibliographic Details
Published in:Blood 2014-07, Vol.124 (4), p.645-653
Main Authors: Jodele, Sonata, Davies, Stella M., Lane, Adam, Khoury, Jane, Dandoy, Christopher, Goebel, Jens, Myers, Kasiani, Grimley, Michael, Bleesing, Jack, El-Bietar, Javier, Wallace, Gregory, Chima, Ranjit S., Paff, Zachary, Laskin, Benjamin L.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Child
Child, Preschool
Female
Follow-Up Studies
Graft vs Host Disease - diagnosis
Graft vs Host Disease - drug therapy
Graft vs Host Disease - etiology
Graft vs Host Disease - mortality
Hematopoietic Stem Cell Transplantation - adverse effects
Humans
Immunosuppressive Agents - therapeutic use
Male
Prognosis
Prospective Studies
Risk Factors
Survival Rate
Thrombotic Microangiopathies - diagnosis
Thrombotic Microangiopathies - drug therapy
Thrombotic Microangiopathies - etiology
Thrombotic Microangiopathies - mortality
Transplantation
Transplantation, Homologous
Young Adult
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Summary:Transplant-associated thrombotic microangiopathy (TMA) leads to generalized endothelial dysfunction that can progress to multiorgan injury, and severe cases are associated with poor outcomes after hematopoietic stem cell transplantation (HSCT). Identifying patients at highest risk for severe disease is challenging. We prospectively evaluated 100 consecutive HSCT recipients to determine the incidence of moderate and severe TMA and factors associated with poor overall outcomes. Thirty-nine subjects (39%) met previously published criteria for TMA. Subjects with TMA had a significantly higher nonrelapse mortality (43.6% vs 7.8%, P < .0001) at 1 year post-HSCT compared with those without TMA. Elevated lactate dehydrogenase, proteinuria on routine urinalysis, and hypertension were the earliest markers of TMA. Proteinuria (>30 mg/dL) and evidence of terminal complement activation (elevated sC5b-9) in the blood at the time of TMA diagnosis were associated with very poor survival (
ISSN:0006-4971
1528-0020
DOI:10.1182/blood-2014-03-564997