ABSTRACT
El síndrome de Evans es un trastorno poco frecuente en el que se observan trombocitopenia y anemia, ambas de etiología autoinmune; las que pueden ocurrir de manera simultánea o sucesiva. Se presenta un caso poco usual de anemia hemolítica autoinmune por anticuerpos fríos asociada a púrpura trombocitopénica autoinmune. Paciente femenina de 22 años de edad con diagnóstico de púrpura trombocitopénica autoinmune, después de 7 años de evolución y un año en remisión, presentó una anemia hemolítica autoinmune por anticuerpos fríos, refractaria al tratamiento con esteroides y alcaloides de la Vinca, que requirió transfusiones de concentrado de eritrocitos y logró la remisión con la administración de anticuerpo monoclonal anti CD 20. Los restantes estudios de autoinmunidad fueron negativos. Actualmente se mantiene asintomática y sin tratamiento inmunosupresor(AU)
Evans syndrome is a rare disorder in which thrombocytopenia and anemia are observed, both of autoimmune aetiology, which may occur simultaneously or successively. A rare case of cold autoimmune hemolytic anemia associated to autoimmune thrombocytopenic purpura is presented. A 22-year-old female patient with diagnosis of autoimmune thrombocytopenic purpura, after 7 years of evolution and one year in remission, has a cold autoimmune hemolytic anemia, refractory to steroid treatment and vinca alkaloids, which requires transfusions of packed erythrocytes and achieves remission with anti CD 20 monoclonal antibody. The remaining studies of autoimmunity are negative. Currently the patient is asymptomatic and without immunosuppressive therapy(AU)
Subject(s)
Humans , Female , Adult , Anemia, Hemolytic, Autoimmune/complications , Purpura, Thrombocytopenic, Idiopathic/diagnosis , Thrombocytopenia/complications , Agglutinins , Rituximab/therapeutic useABSTRACT
Se describe el caso clínico de un adolescente de 16 años de edad, con antecedentes de buena salud, que fue ingresado en la Unidad de Cuidados Intensivos Pediátricos del Hospital Infantil Norte Dr Juan de la Cruz Martínez Maceira de Santiago de Cuba por sospecharse la presencia de neumonía bacteriana y anemia moderada. Los resultados de los exámenes complementarios permitieron concluir que el paciente presentaba anemia hemolítica autoinmune por anticuerpos fríos y anemia falciforme de aparición tardía, por lo cual fue trasladado al Servicio de Hematología, de donde egresó sin complicaciones
The case report of a 16 year-old adolescent , with history of good health is described. He was admitted in the Pediatric Intensive Cares Unit of Dr Juan of the Cruz Martínez Maceira Northern Children Hospital of Santiago de Cuba due to suspicions of the presence of bacterial pneumonia and moderate anemia. The results of the additional tests allowed to conclude that the patient presented autoimmune hemolytic state due to cold antibodies and sickle cell anemia of late onset, reason why he was referred to the Hematology Service, where he was discharged without complications
Subject(s)
Humans , Male , Adolescent , Anemia, Hemolytic , Anemia, Sickle Cell , Secondary Care , Sickle Cell TraitABSTRACT
The purpose of this study was to review the clinical hematological, immunological features and treatment responsiveness in children with autoimmune hemolytic anemia (AHA). Eight children with AHA and positive Coombs' test was evaluated. Seven patients presented with acute onset of symptoms and histories of infection. One case was diagnosed as Evans syndrome, one as a chromosomal anomaly, and one case was combined with the Guillain-Barre syndrome. Among 8 the patients, 4 exhibited warm antibodies and the remainder had cold antibodies. The patients were given washed packed red blood cells, prednisolone or immunosuppressive drugs (6-MP or cyclophosphamide). Five patients responded well to transfusion and/or prednisolone, one patient died and one patient showed no response in 5 months of follow up.