Langerhans cell histiocytosis associated with partial DiGeorge syndrome in a newborn.
OBJECTIVE
We report the unrecognized association of Langerhans cell histiocytosis (LCH) with partial DiGeorge syndrome.


METHODS
A 7-week-old infant with endocrine and immunologic characteristics of DiGeorge syndrome displayed multisystem involvement of Letterer-Siwe disease at birth.


RESULTS
Despite vigorous medical support and chemotherapy, she died at 9 months of age with multisystem failure.


CONCLUSIONS
This case supports the role of the thymus n the pathogenesis of LCH.
Levendoglu-Tugal
O
O
Department of Pediatrics, New York Medical College, Valhalla, USA.
Noto
R
R
Juster
F
F
Brudnicki
A
A
Slim
M
M
Beneck
D
D
Jayabose
S
S
D004062:DiGeorge Syndrome / Q000150:complications*; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D013950:Thymus Gland / Q000503:physiopathology