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Blood, 8 October 2009, Vol. 114, No. 15, pp. 3167-3172. Prepublished online as a Blood First Edition Paper on July 28, 2009; DOI 10.1182/blood-2009-04-215368. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2009-04-215368v1 114/15/3167    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via CrossRef Google Scholar Articles by Michel, M. Articles by Rodeghiero, F. PubMed PubMed Citation Articles by Michel, M. Articles by Rodeghiero, F. Related Collections Immunobiology Free Research Articles Platelets and Thrombopoiesis Red Cells, Iron, and Erythropoiesis Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases Marc Michel1, Valérie Chanet2, Agnès Dechartres3, Anne-Sophie Morin1, Jean-Charles Piette4, Lorenzo Cirasino5, Giovanni Emilia6, Francesco Zaja7, Marco Ruggeri8, Emmanuel Andrès9, Philippe Bierling10, Bertrand Godeau1, and Francesco Rodeghiero8 1 Department of Internal Medicine, Henri Mondor Hospital, Assistance Publique Hôpitaux de Paris, Paris XII University, Créteil, France; 2 University Hospital, Clermont Ferrand, France; 3 Inserm U738, Bichat Hospital, Paris VII University, Paris, France; 4 Department of Internal Medicine, Groupe Hospitalier Pitiè-Salpetrière, Paris, France; 5 Department of Internal Medicine, Niguarda Hospital, Milan, Italy; 6 Department of Hematology/Oncology, University of Modena and Reggio Emilia, Modena, Italy; 7 Clinic of Hematology, Azienda Ospedaliera Universitaria, Udine, Italy; 8 Division of Hematology, S Bortolo Hospital, Vicenza, Italy; 9 Department of Internal Medicine, University Hospital, Strasbourg, France; and 10 Etablissement Français du Sang, Paris-Ile de France, France Evans syndrome (ES) is a rare disease characterized by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) and/or immune neutropenia. To better describe the characteristics and outcome of ES in adults, a survey was initiated in 2005. The data from 68 patients (60% of them women) fulfilling strict inclusion criteria for ES are reported. The mean age at time of ITP and/or AIHA onset was 52 plus or minus 33 years, both cytopenias occurred simultaneously in 37 cases (54.5%). ES was considered as "primary" in 34 patients (50%) but was associated with an underlying disorder in half of the cases, including mainly systemic lupus, lymphoproliferative disorders, and common variable immunodeficiency. All patients were given corticosteroids, but 50 of them (73%) required at least one "second-line" treatment, including splenectomy(n = 19) and rituximab (n = 11). At time of analysis, after a mean follow-up of 4.8 years, only 22 patients (32%) were in remission off treatment; 16 (24%) had died. In elderly patients, the risk of cardiovascular manifestations related to AIHA seems to be higher than the ITP-related risk of severe bleeding. In conclusion, ES is a potentially life-threatening condition that may be associated with other underlying autoimmune or lymphoproliferative disorders. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

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