The European Survey on Adult’s Evans Syndrome: Analysis of 54 Cases.

Evans syndrome (ES) is a rare disorder defined by the combination of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP), both conditions occurring either simultaneously or sequentially. To better describe the main features and outcome of ES in adults, a European survey is ongoing and the first 54 reviewed cases are reported here. AHAI was defined by a haemoglobin level (Hb) ≤ 10g/dL, with features of hemolysis and a positive direct antiglobulin test. ITP was defined according to the usual criteria set for ASH and a platelet count (plt ct) < 100 x 10⁹/L on at least two separate occasions a week apart. Patients with "secondary" ITP were not excluded. Thirty four women (63%) and 20 men (37%) were included. The mean age was respectively 54.7±21, and 54.6±22 years at ITP and AHAI onset. Both conditions occurred simultaneously in 57% of the cases whereas ITP preceded AHAI in 24%; the mean delay between both conditions was 37±35 months. The mean Hb level at diagnosis was 7.7±0.2 g/dL, the mean plt ct was 33x10⁹/L. Bleeding manifestations were present at ITP onset in 28/54 cases (52%) among whom 5 (3.7%) had a life-threatening haemorrhage; 56% of the patients (pts) required at least one transfusion for AHAI at onset. AHAI lead to a cardiac complication (acute coronary syndrome in 4 cases, myocardial infarction in 2) in 6 of the 28 pts (21%) aged of 60 or above. ES was considered as “idiopathic” in 33/54 cases (61%) whereas an underlying disease was found in 21 cases (39%): Systemic lupus or “lupus-like syndrome” (n=10), common variable immunodeficiency (n=3), Non-Hodgkin lymphoma (NHL) (n=2), miscellaneous (n=6). After a median follow-up (f.u) of 3.5 years, a B-cell NHL occurred in 2 other pts and a myelodysplastic syndrome (MDS) was diagnosed in 2, all aged above 72 years. All pts but 2 received steroids as first-line therapy (initial response rate = 80%), 26/54 pts (48%) got intravenous immunoglobulins for ITP (short-term response rate = 62%). Thirty four pts (62%) required at least one other therapy for the management of ITP and/or AIHA including: danazol (n=16), vinca alkaloids (n=7), cyclophosphamide (n=6), azathriopine (n=4), ciclosporin (n=4), mycophenolate (n=1). Fourteen pts underwent splenectomy for corticodepedent AIHA(n=8) or refractory ITP (n=6) leading to a lasting response in 8/15 (57%), a fatal overwhelming postsplenectomy infection occurred in 2 pts. Rituximab was given in 3 pts for refractory ITP leading to 3 complete responses. At time of analysis, after a median f.u of 36/42 months after AIHA/ITP onset, 28 pts (52%) were in remission on treatment (prednisone ± immunosuppressor), 20 (37%) were in treatment-free remission, 6 were not in remission and 12 (22%) had died (sepsis = 5, MDS = 2, other = 5). In conclusion, ES is mainly associated with SLE in young adults and can precede the onset of NHL or MDS in elderly pts. In elderly pts, the risk of cardiac complications related to AIHA seems to be higher than the risk of bleeding related to ITP. For the management of ES, both the severity of the disease and the risk of infection must be taken into consideration.