A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge
Joint Authors
Hashmi, Hamza
Murray, Drew
Greenwell, John
Shaikh, Marwan
Basu, Soumit
Krem, Maxwell
Source
Issue
Vol. 2018, Issue 2018 (31 Dec. 2018), pp.1-6, 6 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2018-05-16
Country of Publication
Egypt
No. of Pages
6
Main Subjects
Abstract EN
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocyte disorder most commonly characterized by multifocal osteosclerotic lesions of the long bones demonstrating sheets of foamy histiocyte infiltrates on biopsy with or without histiocytic infiltration of extraskeletal tissues.
ECD can be difficult to diagnose since it is a very rare disease that can affect many organ systems.
Diagnosis is based on the pathologic evaluation of involved tissue interpreted within the clinical context.
Patients who have the BRAF V600E mutation are treated first line with vemurafenib.
For those without the mutation with symptomatic ECD, conventional or PEGylated interferon alpha is recommended.
For patients who are either intolerant or nonresponsive to interferon alpha, systemic chemotherapy with or without corticosteroids can be used.
We present a rare case of Erdheim-Chester disease with concurrent Langerhans cell histiocytosis which occurs in only one fifth of the cases and often presents as a diagnostic and therapeutic challenge.
American Psychological Association (APA)
Hashmi, Hamza& Murray, Drew& Greenwell, John& Shaikh, Marwan& Basu, Soumit& Krem, Maxwell. 2018. A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge. Case Reports in Hematology،Vol. 2018, no. 2018, pp.1-6.
https://search.emarefa.net/detail/BIM-1144020
Modern Language Association (MLA)
Hashmi, Hamza…[et al.]. A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge. Case Reports in Hematology No. 2018 (2018), pp.1-6.
https://search.emarefa.net/detail/BIM-1144020
American Medical Association (AMA)
Hashmi, Hamza& Murray, Drew& Greenwell, John& Shaikh, Marwan& Basu, Soumit& Krem, Maxwell. A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge. Case Reports in Hematology. 2018. Vol. 2018, no. 2018, pp.1-6.
https://search.emarefa.net/detail/BIM-1144020
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1144020