Experience with Evans syndrome in an academic referral center

Objective: To 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. Methods: Patients enrolled in this study fulfilled criteria for Evans syndrome. Data were retrieved f...

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Autores principales: Jaime Pérez, José Carlos, Guerra Leal, Liliana Nataly, López Razo, Olga Nidia, Méndez Ramírez, Nereida, Gómez Almaguer, David
Formato: Artículo
Lenguaje:inglés
Publicado: 2015
Acceso en línea:http://eprints.uanl.mx/14983/1/145.pdf
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author Jaime Pérez, José Carlos
Guerra Leal, Liliana Nataly
López Razo, Olga Nidia
Méndez Ramírez, Nereida
Gómez Almaguer, David
author_facet Jaime Pérez, José Carlos
Guerra Leal, Liliana Nataly
López Razo, Olga Nidia
Méndez Ramírez, Nereida
Gómez Almaguer, David
author_sort Jaime Pérez, José Carlos
collection Repositorio Institucional
description Objective: To 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. Methods: Patients 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. Results: Six 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. Conclusion: Evans syndrome is an uncommon hematologic condition rarely diagnosed and not widely studied. Clinicians must have itin 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.
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spelling eprints-149832019-05-10T17:37:31Z http://eprints.uanl.mx/14983/ Experience with Evans syndrome in an academic referral center Jaime Pérez, José Carlos Guerra Leal, Liliana Nataly López Razo, Olga Nidia Méndez Ramírez, Nereida Gómez Almaguer, David Objective: To 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. Methods: Patients 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. Results: Six 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. Conclusion: Evans syndrome is an uncommon hematologic condition rarely diagnosed and not widely studied. Clinicians must have itin 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. 2015 Article PeerReviewed text en cc_by_nc_nd http://eprints.uanl.mx/14983/1/145.pdf http://eprints.uanl.mx/14983/1.haspreviewThumbnailVersion/145.pdf Jaime Pérez, José Carlos y Guerra Leal, Liliana Nataly y López Razo, Olga Nidia y Méndez Ramírez, Nereida y Gómez Almaguer, David (2015) Experience with Evans syndrome in an academic referral center. Revista Brasileira de Hematologia e Hemoterapia, 37 (4). pp. 230-235. ISSN 15168484 http://doi.org/10.1016/j.bjhh.2015.03.002 doi:10.1016/j.bjhh.2015.03.002
spellingShingle Jaime Pérez, José Carlos
Guerra Leal, Liliana Nataly
López Razo, Olga Nidia
Méndez Ramírez, Nereida
Gómez Almaguer, David
Experience with Evans syndrome in an academic referral center
thumbnail https://rediab.uanl.mx/themes/sandal5/images/online.png
title Experience with Evans syndrome in an academic referral center
title_full Experience with Evans syndrome in an academic referral center
title_fullStr Experience with Evans syndrome in an academic referral center
title_full_unstemmed Experience with Evans syndrome in an academic referral center
title_short Experience with Evans syndrome in an academic referral center
title_sort experience with evans syndrome in an academic referral center
url http://eprints.uanl.mx/14983/1/145.pdf
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