Abstract
Thrombotic microangiopathy (TMA) is a grave complication after haematopoietic stem cell transplantation (HSCT) and effective treatment is undefined. Five patients with postHSCT TMA, which was refractory to at least 1 week of plasma exchange and prednisolone, were treated with rituximab (375 mg/m(2)/week x 4). Remission was achieved in four patients, of whom three remained in remission and one had died of sepsis at a median follow-up of 10 months. ADAMTS13 levels were low in all evaluable patients, and only one patient showed significant anti-ADAMTS13 antibody. The levels of ADAMTS13 and anti-ADAMTS13 antibody did not change significantly with rituximab-induced remission.
MeSH terms
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ADAM Proteins / immunology
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ADAMTS13 Protein
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Adult
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Antibodies, Monoclonal / therapeutic use*
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Antibodies, Monoclonal, Murine-Derived
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Autoantigens / blood
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Biomarkers / blood
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Female
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Glucocorticoids / therapeutic use
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Hematopoietic Stem Cell Transplantation / adverse effects*
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Humans
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Immunologic Factors / therapeutic use*
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Infant
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Male
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Microcirculation
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Middle Aged
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Plasma Exchange
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Prednisolone / therapeutic use
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Remission Induction
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Retrospective Studies
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Rituximab
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Thrombosis / drug therapy*
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Thrombosis / etiology*
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Thrombosis / therapy
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Treatment Outcome
Substances
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Antibodies, Monoclonal
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Antibodies, Monoclonal, Murine-Derived
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Autoantigens
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Biomarkers
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Glucocorticoids
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Immunologic Factors
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Rituximab
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Prednisolone
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ADAM Proteins
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ADAMTS13 Protein
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ADAMTS13 protein, human