Document Detail

Presence of Erdheim-Chester Disease and Langerhans Cell Histiocytosis in the Same Patient: A Report of 2 Cases.
MedLine Citation:
PMID:  21326112     Owner:  NLM     Status:  Publisher    
The histiocytic disorders Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD), can both present with multisystem involvement, with the central nervous system and the bone, skin, neuroendocrine, cardiac, respiratory, and gastrointestinal systems potentially affected. The 2 entities occasionally can be difficult to distinguish. Both rarely affect the orbit and the central nervous system, and although there are rare reports of patients with coexistent LCH and ECD, there are no reported cases of the 2 diseases that involve both the orbital and neuroendocrine systems. We report 2 such cases, and review the literature of cases of LCH and ECD occurring in the same patient. The presentation of LCH and ECD in certain patients suggests a possible abnormality in the common CD34 progenitor cell. The coexistence of the 2 disease states should be suspected in patients with atypical presentations of either disorder.
Stacy L Pineles; Grant T Liu; Xenia Acebes; Jorge Arruga; Sunita Nasta; Ruchira Glaser; Michelle Pramick; Franz Fogt; Peter Le Roux; Roberta E Gausas
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-2-14
Journal Detail:
Title:  Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society     Volume:  -     ISSN:  1536-5166     ISO Abbreviation:  -     Publication Date:  2011 Feb 
Date Detail:
Created Date:  2011-2-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9431308     Medline TA:  J Neuroophthalmol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Division of Neuro-Ophthalmology, Department of Neurology (SLP, GTL), Departments of Ophthalmology (SLP, GTL, REG), Hematology-Oncology (SN), Cardiology (RG), Pathology (FF), and Neurosurgery (PLR), Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania; Departments of Neurology and Ophthalmology (XA, JA), Bellvitge Hospital, Barcelona, Spain; and Department of Pathology (MP), Pennsylvania Hospital, Philadelphia, Pennsylvania.
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