Langerhans cell histiocytosis associated with partial DiGeorge syndrome in a newborn

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. Conclusion: This case supports the role of the thymus in the pathogenesis of LCH.