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J Pediatr Hematol Oncol ; 42(8): e745-e749, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-31568177

RESUMO

Primary immunodeficiencies are inherited disorders, which may be revealed in the context of autoimmune hemolytic anemia (AIHA). We report the case of a girl presenting with an enterovirus-related AIHA. Despite being in complete remission for her anemia after treatment, the initial CD4/CD8 lymphopenia dramatically worsened with time. Its sole clinical presentation was generalized verrucosis. Cellular quantitative and functional immunodeficiency was evidenced but no known molecular defect was identified despite extensive workup. This unlabeled profound naive T-lymphopenia was cured by bone marrow transplantation. No similar case was ever described in the scientific literature. Patients with AIHA and/or generalized verrucosis should be screened for primary immunodeficiency, before initiating any immunomodulatory treatment.


Assuntos
Anemia Hemolítica Autoimune/tratamento farmacológico , Elefantíase/patologia , Linfopenia/patologia , Esteroides/efeitos adversos , Linfócitos T/imunologia , Anemia Hemolítica Autoimune/patologia , Criança , Terapia Combinada , Elefantíase/induzido quimicamente , Elefantíase/terapia , Feminino , Humanos , Linfopenia/induzido quimicamente , Linfopenia/terapia , Prognóstico , Linfócitos T/efeitos dos fármacos
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