Patient 1 was the only case in which steroids were successfully used as both first-line treatment and during relapses without additional medications; in all the remaining five cases a combination of therapies was needed to achieve response. Response times, as days needed for the increase in hemoglobin concentration and platelet count to take place, are shown in Table 3.
Patients were discharged at a median of nine days range: days. Relapse was diagnosed when patients presented with same or similar symptoms as baseline and the simultaneous or sequential presence of a positive anti-globulin test, autoimmune hemolytic anemia and immune thrombocytopenia.
An appropriate follow-up was documented in five of the six patients. Three patients relapsed within nine months to five years. One patient Patient 2 responded satisfactorily to initial steroid therapy and was subsequently lost to follow-up.
Two patients, 5 and 6, had not relapsed 45 and 32 months after the initial diagnosis, respectively. Patients 1, 3, and 4 relapsed. Patient 1 presented two relapses, the first one, five years after diagnosis, was treated with steroids, obtaining complete remission until a year later when she presented moderate thrombocytopenia and severe AIHA. This episode was again treated as in her first relapse, with steroids, obtaining a third remission lasting 24 months to date.
Patient 3 had a relapse at ten months after diagnosis; she was treated with dexamethasone, prednisone and rituximab, with no response; splenectomy was performed at this time. This patient has not relapsed to date, 57 months after splenectomy. Patient 4 relapsed at nine months. She was treated with splenectomy and has not suffered further relapse 32 months after removal of the spleen; clinical course and therapy for these five patients are summarized in Table 3.
Evans syndrome is a rare autoimmune regulation disorder whose exact pathophysiology is unknown. Savasan et al. Wang et al. Decreases in the production of interleukin and gamma-interferon have been reported, and it is postulated that this causes the activation of B cells producing auto-antibodies.
These alterations are not exclusive to Evans syndrome as they can be found in other immune diseases, thus it remains to be established if they are indeed a cause of the disease or merely associated immune phenomena.
Its exact incidence remains unknown. In a review of adult patients with immunocytopenias including patients with cases of AIHA and cases of thrombocytopenia, Evans syndrome was diagnosed in only six 0. Acquired hemolytic anemia and associated thrombocytopenic purpura with special reference to Evans syndrome. Proc Staff Meet Mayo Clin. Evans syndrome in childhood. J Pediatr. Evan's Syndrome. J Med. In our study five of six patients had an autoimmune disease diagnosed before they developed Evans syndrome.
The disease is characterized by frequent exacerbations and remissions within a chronic course and response to treatment varies even within the same individual. Most patients require treatment although occasional spontaneous remissions have been reported, as was the case in one of 42 patients. Evans syndrome results of a national survey. J Pediatr Hematol Oncol. Indications for treatment have not been established by other evidence-based studies.
However, it is reasonable and usual to treat symptomatic patients with low blood counts; not all asymptomatic patients with low counts require treatment and the decision to treat or not should be considered according to each individual case. Most of the data are anecdotal and inconclusive with difficult interpretation because of the concomitant use of corticosteroids and other treatment modalities.
Evans syndrome in childhood pathophysiology, clinical course, and treatment. Am J Pediatr Hematol Oncol. Corticosteroids remain the mainstay of therapy for control of the acute symptomatic cytopenias, with good initial results, despite lack of controlled trials demonstrating their efficacy.
Table 5 Proposals for a minimal work-up in adults with a newly diagnosed ES. Primary thrombocytopenic purpura and acquired haemolytic anemia; evidence for a common etiology. Acquired hemolytic anemia; the relation of erythrocyte antibody production to activity of the disease; the significance of thrombocytopenia and leukopenia. Search ADS. Alarcon-Segovia D. Occurrence of both hemolytic anemia and thrombocytopenic purpura Evans' syndrome in systemic lupus erythematosus.
Relationship to antiphospholipid antibodies. Autoimmune thrombocytopenic purpura and common variable immunodeficiency: analysis of 21 cases and review of literature.
Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome ALPS. Idiopathic thrombocytopenic purpura: a practice guide developed by explicit methods for the American Society of Hematology.
Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus [letter]. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome APS.
Results of a prospective concerted action supported by the European Community. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura ITP of adults and children: report from an International Working Group. Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium.
Treatment of splenic marginal zone B-cell lymphoma: an analysis of 81 patients. The incidence of idiopathic thrombocytopenic purpura in adults increases with age. Evans' syndrome: a retrospective study from the ship French Society of Pediatric Hematology and Immunology 36 cases [in French]. Is splenectomy still the gold standard for the treatment of chronic ITP? The efficacy and safety of B-cell depletion with anti-CD20 monoclonal antibody in adults with chronic immune thrombocytopenic purpura.
Efficacy and safety of rituximab in adults' warm antibody autoimmune haemolytic anemia: retrospective analysis of 27 cases.
Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune haemolytic anemia and Evans syndrome. Control of steroid-resistant autoimmune haemolytic anemia by cyclosporine. Long-term salvage treatment by cyclosporin in refractory autoimmune haematological disorders. Mycophenolate mofetil for the treatment of refractory autoimmune haemolytic anemia and autoimmune thrombocytopenic purpura. Efficacy of mycophenolate mofetil in adult refractory auto-immune cytopenias: a single center preliminary study.
Add comment Close comment form modal. Submit a comment. Comment title. You have entered an invalid code. Submit Cancel. Thank you for submitting a comment on this article. Your comment will be reviewed and published at the journal's discretion. For Patients. Evans Syndrome. Contact the Blood Disorders Center International Email hemeclinic-dl childrens. It was also possible to diagnose active SLE due to the presence of clinical criteria hemolytic anemia, thrombocytopenia, pericarditis and oral ulcers.
After pulse therapy, there was clinical improvement, but thrombocytopenia persisted Table 5. The patient was discharged and initiated rheumatologic disease outpatient follow-up. In February he started using azathioprine, obtaining stabilization of platelet levels and remaining without anemia. ES is diagnosed in only 0. Few data are available in the literature; the majority is from pediatric cases.
Treatment strategies frequently used for SLE contribute to greater likelihood of remission of the disease and fewer exacerbation than observed in the general population with ES 8 8 Michel M, Chanet V, Dechartres A, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood [Internet].
There is still no consensus on which laboratory tests and radiological procedures should be performed to search for an underlying disease. A case of Evans syndrome associated with autoimmune thyroiditis.
Hematol Transfus Int J. There are no systemic or randomized studies on ES treatment, which remains a challenge. The therapeutic methods besides glucocorticoids, IVIG and splenectomy reported as successful in some patients were: rituximab, cyclophosphamide, mycophenolate mofetil, cyclosporine, vincristine, danazol, haematopoietic stem cell transplantation and azathioprine 6 6 Schrier SL. Abrir menu Brasil. Jornal Brasileiro de Patologia e Medicina Laboratorial.
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