📇 Rhumatologue ·
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Praticien-chercheur
17 articles scientifiques publiés — formation continue solide
✨ Génération du profil synthétique IA en cours…
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Pediatrics · 2003
Objectives. To evaluate the occurrence of autoimmune and inflammatory complications in Wiskott-Aldrich syndrome (WAS) and to determine risk factors and the prognosis of such complications with the aim of improving the definition of treatment options.Methods. We reviewed the records of 55 patients with WAS evaluated at Necker-Enfants Malades Hospital (Paris) from 1980 to 2000.Results. Forty patients (72%) had at least 1 autoimmune or inflammatory complication. Autoimmune hemolytic anemia was detected in 20 cases (36%); in all cases, onset occurred before the age of 5 years. Other complications included neutropenia (25%), arthritis (29%), skin vasculitis (22%), cerebral vasculitis (7%), inflammatory bowel disease (9%), and renal disease (3%). The median survival of the entire population was 14.5 years. Two autoimmune complications and 1 biological factor were predictive of a poor prognosis in this population: autoimmune hemolytic anemia, severe thrombocytopenia recurring after splenectomy, and high serum immunoglobulin M (IgM) levels before splenectomy. Autoimmune hemolytic anemia was significantly more observed in patients with high serum IgM level.Conclusions. High serum IgM concentration before splenectomy was identified as a risk factor for autoimmune hemolytic anemia; however, it must be confirmed. Autoimmune hemolytic anemia and severe thrombocytopenia recurring after splenectomy were 2 indicators of a poor prognosis. Those results suggest that patients with WAS and IgM levels more than mean + 2 standard deviations before splenectomy should be placed under strict surveillance. Furthermore, severe autoimmune complications should lead, as early as possible, to hematopoietic stem cell transplantation using the best available donor.
Seminars in hematology · 2004
Source PubMed · Recherche par auteur (homonymes possibles, vérifier l'affiliation).
Hemoglobin · 2009 · Case Reports
Favale F, Gardembas M, Pajot O, Saada V, et al.
Blood cells, molecules & diseases · 2007 · Journal Article
Proust A, Guillet B, Picard C, de Saint Basile G, et al.
European journal of pediatrics · 2006 · Case Reports
Picard C, Mellouli F, Duprez R, Chédeville G, et al.
Blood · 2006 · Case Reports
Beaumont C, Delaunay J, Hetet G, Grandchamp B, et al.
European journal of haematology · 2005 · Case Reports
Proust A, Guillet B, Pellier I, Rachieru P, et al.
British journal of haematology · 2004 · Case Reports
Fricke B, Jarvis HG, Reid CD, Aguilar-Martinez P, et al.
Pediatrics · 2003 · Journal Article
Dupuis-Girod S, Medioni J, Haddad E, Quartier P, et al.
European journal of haematology · 2011 · Journal Article
Jeanson-Leh L, Charrier S, Proust A, Bilhou-Nabéra C, et al.
American journal of hematology · 2010 · Journal Article
Crétien A, Proust A, Delaunay J, Rincé P, et al.
European journal of haematology · 2007 · Journal Article
Beaurain G, Mathieu F, Grootenboer S, Fiquet B, et al.
Pediatric blood & cancer · 2011 · Case Reports
Chandrakasan S, Singh S, Dogra S, Delaunay J, et al.
Pediatric hematology and oncology · 2006 · Journal Article
Saada V, Cynober T, Brossard Y, Schischmanoff PO, et al.
Journal of pediatric hematology/oncology · 2005 · Journal Article
Bader-Meunier B, Leverger G, Tchernia G, Schischmanoff O, et al.
Pediatric hematology and oncology · 2006 · Journal Article
Saada V, Cynober T, Brossard Y, Schischmanoff PO, et al.
Seminars in hematology · 2004 · Journal Article
Delaunay J
The hematology journal : the official journal of the European Haematology Association · 2003 · Journal Article
Delaunay J
Pediatric research · 2003 · Case Reports
Zdebska E, Bader-Meunier B, Schischmanoff PO, Dupré T, et al.
Journal of pediatric hematology/oncology · 2002 · Case Reports
Roda L, Pasche J, Fournier A, Terorotua V, et al.