RESUMO
Ataxia-telangiectasia is an autosomal recessive disease characterized by progressive cerebellar ataxia, oculocutaneous telangiectasia, immunodeficiency, high incidence of cancer, and increased sensitivity to ionizing radiation. The authors report a case of dysgerminoma in a child with high alpha-fetoprotein, CA125 and beta-human chorionic gonadotropin, who has been followed-up for ataxia-telangiectasia for 2 years.
Assuntos
Ataxia Telangiectasia/complicações , Disgerminoma/etiologia , Neoplasias Ovarianas/etiologia , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ataxia Telangiectasia/sangue , Ataxia Telangiectasia/terapia , Biomarcadores Tumorais/sangue , Bleomicina/administração & dosagem , Bleomicina/efeitos adversos , Antígeno Ca-125/sangue , Carboplatina/administração & dosagem , Criança , Gonadotropina Coriônica Humana Subunidade beta/sangue , Disgerminoma/tratamento farmacológico , Disgerminoma/cirurgia , Etoposídeo/administração & dosagem , Feminino , Seguimentos , Predisposição Genética para Doença , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Proteínas de Neoplasias/sangue , Neoplasias Ovarianas/tratamento farmacológico , Neoplasias Ovarianas/cirurgia , Ovariectomia , Transtornos Respiratórios/induzido quimicamente , alfa-Fetoproteínas/análiseRESUMO
Autoimmune hemolytic anemia (AIHA) is an unusual complication of malignancy. We diagnosed primary splenic anaplastic large cell lymphoma (ALCL) in a patient. A seven-year-old boy presented with Coombs test-positive hemolytic anemia. After a course of prednisolone therapy, a complete response for anemia was achieved. Twenty months later, in addition to severe hemolytic anemia, the patient was diagnosed with ALCL after splenectomy and pathologic examination of the sample. The recognition of this clinical picture as a complication of non-Hodgkin's lymphoma has important implications. The most effective management of AIHA in the setting of cancer is to treat the underlying malignancy.