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Langerhans cell histiocytosis associated with partial DiGeorge syndrome in a newborn
O Levendoglu-Tugal1, R Noto, F Juster
1Department of Pediatrics, New York Medical College, Valhalla, USA.
Journal of Pediatric Hematology/Oncology
|November 1, 1996
Abstract
Purpose:
We report the unrecognized association of Langerhans cell histiocytosis (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 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.