A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge
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...
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| Format: | Article |
| Language: | English |
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Wiley
2018-01-01
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| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2018/7865325 |
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| author | Hamza Hashmi Drew Murray John Greenwell Marwan Shaikh Soumit Basu Maxwell Krem |
| author_facet | Hamza Hashmi Drew Murray John Greenwell Marwan Shaikh Soumit Basu Maxwell Krem |
| author_sort | Hamza Hashmi |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-94f2a3bf27514964a76534b2b2f2c32e |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-94f2a3bf27514964a76534b2b2f2c32e2025-02-03T01:02:47ZengWileyCase Reports in Hematology2090-65602090-65792018-01-01201810.1155/2018/78653257865325A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic ChallengeHamza Hashmi0Drew Murray1John Greenwell2Marwan Shaikh3Soumit Basu4Maxwell Krem5Division of Hematology and Oncology, University of Louisville, Louisville, KY, USADivision of Hematology and Oncology, University of Louisville, Louisville, KY, USADivision of Pathology and Laboratory Medicine, University of Louisville, Louisville, KY, USADivision of Hematology and Oncology, University of West Virginia, Morgantown, WV, USAGeisinger Health System, Center for Blood and Marrow Transplant, Danville, PA, USADivision of Blood and Marrow Transplant, University of Louisville, Louisville, KY, USAErdheim-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.http://dx.doi.org/10.1155/2018/7865325 |
| spellingShingle | Hamza Hashmi Drew Murray John Greenwell Marwan Shaikh Soumit Basu Maxwell Krem 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 |
| title | A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge |
| title_full | A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge |
| title_fullStr | A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge |
| title_full_unstemmed | A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge |
| title_short | A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge |
| title_sort | rare case of erdheim chester disease non langerhans cell histiocytosis with concurrent langerhans cell histiocytosis a diagnostic and therapeutic challenge |
| url | http://dx.doi.org/10.1155/2018/7865325 |
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