title: The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Henri Mondor Hospital, Assistance Publique Hôpitaux de Paris, Paris XII University, Créteil, France. marc.michel@hmn.aphp.fr
authors.forename: Marc
authors.initials: M
authors.lastname: Michel
authors.forename: Valérie
authors.initials: V
authors.lastname: Chanet
authors.forename: Agnès
authors.initials: A
authors.lastname: Dechartres
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: Morin
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Cirasino
authors.forename: Giovanni
authors.initials: G
authors.lastname: Emilia
authors.forename: Francesco
authors.initials: F
authors.lastname: Zaja
authors.forename: Marco
authors.initials: M
authors.lastname: Ruggeri
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Andrès
authors.forename: Philippe
authors.initials: P
authors.lastname: Bierling
authors.forename: Bertrand
authors.initials: B
authors.lastname: Godeau
authors.forename: Francesco
authors.initials: F
authors.lastname: Rodeghiero
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000367:Age Factors; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000209:etiology / Q000401:mortality* / Q000628:therapy*; D000911:Antibodies, Monoclonal / Q000008:administration & dosage; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000008:administration & dosage; D003625:Data Collection; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009503:Neutropenia / Q000175:diagnosis / Q000209:etiology / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000175:diagnosis / Q000209:etiology / Q000401:mortality* / Q000628:therapy*; D012074:Remission Induction; D012189:Retrospective Studies; D012307:Risk Factors; D000069283:Rituximab; D013156:Splenectomy / Q000379:methods; D015996:Survival Rate; D013577:Syndrome
chemical_list: D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab