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Case Reports in Hematology
Volume 2015, Article ID 670843, 4 pages
http://dx.doi.org/10.1155/2015/670843
Case Report

Langerhans Cell Histiocytosis in an Infant Mimicking a Lymphoma at Presentation

1Pediatric Hematology and Oncology Unit, Dubai Hospital, Dubai, UAE
2Radiology Department, Dubai Hospital, Dubai, UAE

Received 3 July 2015; Revised 9 October 2015; Accepted 13 October 2015

Academic Editor: Yusuke Shiozawa

Copyright © 2015 Anjan Madasu et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation and accumulation of clonal dendritic cells with varied clinical presentation and an unpredictable course. We report a 5-month-old infant with LCH who presented with severe respiratory distress, a large mediastinal mass, significant generalized lymphadenopathy, and hepatosplenomegaly. Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy. However, lack of response prompted a biopsy which confirmed it to be a case of LCH and that leads to appropriate therapy and survival. There have been reports of LCH presenting with isolated mediastinal mass or with generalized lymphadenopathy, but the combined presentation of generalized lymphadenopathy with large mediastinal mass, hepatosplenomegaly, and fever in an infant has rarely been reported. Conclusion. LCH should also be considered in the differential diagnosis of an infant presenting with generalized lymphadenopathy, mediastinal mass, hepatosplenomegaly, and fever.