Langerhans cell histiocytosis associated with partial DiGeorge syndrome in a newborn.

作者信息

Levendoglu-Tugal O, Noto R, Juster F, Brudnicki A, Slim M, Beneck D, Jayabose S

机构信息

Department of Pediatrics, New York Medical College, Valhalla, USA.

出版信息

J Pediatr Hematol Oncol. 1996 Nov;18(4):401-4. doi: 10.1097/00043426-199611000-00014.

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.