The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases.
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.
Michel
Marc
M
Department of Internal Medicine, Henri Mondor Hospital, Assistance Publique Hôpitaux de Paris, Paris XII University, Créteil, France. marc.michel@hmn.aphp.fr
Chanet
Valérie
V
Dechartres
Agnès
A
Morin
Anne-Sophie
AS
Piette
Jean-Charles
JC
Cirasino
Lorenzo
L
Emilia
Giovanni
G
Zaja
Francesco
F
Ruggeri
Marco
M
Andrès
Emmanuel
E
Bierling
Philippe
P
Godeau
Bertrand
B
Rodeghiero
Francesco
F
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
D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab