Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy

Summary Background Thrombotic thrombocytopenic purpura is a thrombotic microangiopathy related to a severe deficiency of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13; activity

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Veröffentlicht in:	The Lancet. Haematology 2016-05, Vol.3 (5), p.e237-e245
Hauptverfasser:	Mariotte, Eric, MD, Azoulay, Elie, Prof, Galicier, Lionel, MD, Rondeau, Eric, Prof, Zouiti, Fouzia, MD, Boisseau, Pierre, PhD, Poullin, Pascale, MD, de Maistre, Emmanuel, MD, Provôt, François, MD, Delmas, Yahsou, MD, Perez, Pierre, MD, Benhamou, Ygal, MD, Stepanian, Alain, PhD, Coppo, Paul, Prof, Veyradier, Agnès, Prof
Format:	Artikel
Sprache:	eng
Schlagworte:	ADAMTS13 Protein - deficiency ADAMTS13 Protein - genetics ADAMTS13 Protein - immunology Adult Autoantibodies - blood Autoimmune Diseases - complications Autoimmune Diseases - etiology Clopidogrel Cohort Studies Cross-Sectional Studies Digestive System Diseases - complications Female Fever - complications France - epidemiology Genotype Hematology, Oncology and Palliative Medicine HIV Infections - complications Humans Infection - complications Male Middle Aged Mutation - genetics Neoplasms - complications Nervous System Diseases - complications Pregnancy Pregnancy Complications - physiopathology Prevalence Purpura, Thrombocytopenic, Idiopathic - complications Purpura, Thrombocytopenic, Idiopathic - epidemiology Purpura, Thrombocytopenic, Idiopathic - genetics Purpura, Thrombocytopenic, Idiopathic - physiopathology Purpura, Thrombotic Thrombocytopenic - complications Purpura, Thrombotic Thrombocytopenic - epidemiology Purpura, Thrombotic Thrombocytopenic - genetics Purpura, Thrombotic Thrombocytopenic - physiopathology Registries Risk Factors Sex Factors Ticlopidine - adverse effects Ticlopidine - analogs & derivatives Transplantation - adverse effects
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Zusammenfassung:	Summary Background Thrombotic thrombocytopenic purpura is a thrombotic microangiopathy related to a severe deficiency of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13; activity
ISSN:	2352-3026 2352-3026
DOI:	10.1016/S2352-3026(16)30018-7