Experience with Evans syndrome in an academic referral center

Abstract

ObjectiveTo document the experience of one referral service with patients diagnosed with Evans syndrome, the treatment and response and to briefly review current treatment strategies and results.MethodsPatients enrolled in this study fulfilled criteria for Evans syndrome. Data were retrieved from the clinical files and electronic databases of the Department of Hematology, Hospital Universitario “Dr. José Eleuterio González”. Treatment modalities and response and the use of additional therapies were evaluated. The literature was reviewed in the context of the clinical course of the studied patients.ResultsSix patients were diagnosed with Evans syndrome in the study period. Patient 1 was treated with steroids, relapsed twice and was again treated with steroids. Patient 2 treated initially with steroids plus intravenous immunoglobulin was subsequently lost to follow-up. A good response was achieved in Patients 3 and 4, who were treated with steroids plus rituximab; patient 4 also received danazol as a second-line therapy. However both relapsed and subsequently underwent splenectomy at ten and nine months, respectively. One patient, number 5, treated with steroids, danazol and rituximab did not relapse within four years of follow-up and Patient 6, who received steroids plus danazol did not relapse within three years of follow-up.ConclusionEvans syndrome is an uncommon hematologic condition rarely diagnosed and not widely studied. Clinicians must have it in mind when evaluating a patient with a positive direct antiglobulin test, anemia and thrombocytopenia, since prognosis depends on its early recognition and opportune therapy, but even this leads to variable results.

Evans syndrome: clinical perspectives, biological insights and treatment modalities

“…Clinical features are associated with anemia and thrombocytopenia including pallor, weakness, fatigue, jaundice, petechiae, ecchymosis, gingivorrhagia and epistaxis. 5 , 8 , 10 Due to the prolonged immunosuppressive therapy and/or the associated underlying immune deficit, there is a risk of 66.6% of patients developing respiratory tract infections. 8 …”

“…Previously named Canale–Smith syndrome, 53 autoimmune lymphoproliferative syndrome (ALPS) is a disorder characterized by a Fas gene mutation with an alteration in T-cell apoptoic pathways causing lymphoproliferation manifested as hepatomegaly, splenomegaly, lymphadenopathies, an increased risk of Hodgkin lymphoma and immune cytopenia, mainly AIHA and ITP, with the presence of antibodies against erythrocytes and platelets, 54 features similar to those observed in autoimmune multilineage cytopenia. 10 Diagnostic criteria for ALPS were established in 1999 by the National Institutes of Health and require two necessary criteria, such as the presence of chronic lymphoproliferation, increased levels of double-negative T-cells and one primary accessory criterion with a deficit of apoptosis in vitro or genetic mutation in FAS, FASL and/or CASP 10. 55 Some authors suggest ruling out this diagnosis in all pediatric patients with AIHA and ES.…”

“…Several studies have demonstrated the relationship between SLE and ES; 9 , 10 , 58 prevalence of secondary ES in SLE has been reported in 1.7%–2.7% of cases. 24 , 59 Hemorrhagic manifestations are frequently observed in this group, including petechiae, ecchymosis, epistaxis, gingivorraghia, gastrointestinal bleeding and hematuria.…”

Scrotal Pyoderma Gangrenosum Associated with Evans Syndrome

“…Dysregulation and abnormalities in cellular and humoral immunity can be found in Evans syndrome. Clinical presentations of Evans syndrome include the usual features of hemolytic anemia (jaundice, pallor, and dizziness) and thrombocytopenia (petechiae and mucocutaneous bleeding) [ 4 ]. There are many secondary causes of Evans syndrome, including systemic lupus erythematosus, primary antiphospholipid syndrome, Sjogren’s syndrome, primary immunodeficiency, lymphoma, leukemia, and lymphoproliferative disorders [ 5 , 6 , 7 ].…”

Синдром на Evans-Fisher при солидни тумори

“…Evans-Fisher е много рядък паранеопластичен синдром при солидните тумори [5]. Въпреки че честотата е неизвестна, изследванията показват по-висок процент при пациенти от женски пол [6]. Клиничният дебют на заболяването може да се демонстрира с нарушения в хематологичните показатели [7].…”