LANGERHANS CELL HISTIOCYTOSIS ASSOCIATED WITH PARTIAL DIGEORGE-SYNDROME IN A NEWBORN

Authors
LEVENDOGLUTUGAL O NOTO R JUSTER F BRUDNICKI A SLIM M BENECK D JAYABOSE S
Citation
O. Levendoglutugal et al., LANGERHANS CELL HISTIOCYTOSIS ASSOCIATED WITH PARTIAL DIGEORGE-SYNDROME IN A NEWBORN, Journal of pediatric hematology/oncology, 18(4), 1996, pp. 401-404
Citations number
27
Categorie Soggetti
Oncology,Hematology,Pediatrics
Journal title
Journal of pediatric hematology/oncology → ACNP
ISSN journal
10774114
Volume
18
Issue
4
Year of publication
1996
Pages
401 - 404
Database
ISI
SICI code
1077-4114(1996)18:4<401:LCHAWP>2.0.ZU;2-Q
Abstract
Purpose: We report the unrecognized association of Langerhans cell his tiocytosis (LCH) with partial DiGeorge syndrome. Patient and Methods: A 7-week-old infant with endocrine and immunologic characteristics of DiGeorge syndrome displayed multisystem involvement of Letterer-Siwe d isease at birth. Results: Despite vigorous medical support and chemoth erapy, she died at 9 months of age with multisystem failure. Conclusio n: This case supports the role of the thymus in the pathogenesis of LC H.