Generalized Verrucosis Revealing a Life-Threatening and Unlabeled T-Cell Lymphopenia Associated With Autoimmune Hemolytic Anemia: A Case Report and Review of Literature.
J Pediatr Hematol Oncol
; 42(8): e745-e749, 2020 11.
Article
em En
| MEDLINE
| ID: mdl-31568177
ABSTRACT
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.
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Esteroides
/
Linfócitos T
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Elefantíase
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Anemia Hemolítica Autoimune
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Linfopenia
Tipo de estudo:
Prognostic_studies
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Risk_factors_studies
Limite:
Child
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Female
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Humans
Idioma:
En
Revista:
J Pediatr Hematol Oncol
Assunto da revista:
HEMATOLOGIA
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NEOPLASIAS
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PEDIATRIA
Ano de publicação:
2020
Tipo de documento:
Article