Concomitant Langerhans and Interdigitating Reticulum Cell Hyperplasia in a Reactive Lymph Node of an Infant with Omenn Syndrome: A Diagnostic Pitfall for Langerhans Cell Histiocytosis.
Int J Surg Pathol
; 31(1): 64-68, 2023 Feb.
Article
em En
| MEDLINE
| ID: mdl-35686332
ABSTRACT
Omenn syndrome, a rare form of combined immunodeficiency in infants, presenting with recurrent infections, erythroderma, lymphadenopathy, hepatosplenomegaly, eosinophilia, and increased serum IgE levels. It is a fatal condition unless treated by hematopoietic stem cell transplant. Hence, an early diagnosis and a prompt treatment can lead to better outcome in these unfortunate babies afflicted with Omenn syndrome. Here, we present an 8-week-old infant with typical features of Omenn syndrome, both clinically as well as on laboratory analysis, but surprising immunohistochemical findings on lymph node biopsy.
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Base de dados:
MEDLINE
Assunto principal:
Histiocitose de Células de Langerhans
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Imunodeficiência Combinada Severa
Idioma:
En
Ano de publicação:
2023
Tipo de documento:
Article