Bibliographic Details
Title: |
Leg‐type form of idiopathic multicentric Castleman disease associated with severe lower extremity chronic venous/lymphatic disease |
Authors: |
Thomas Ballul, Nabil Belfeki, Adèle deMasson, Véronique Meignin, Paul‐Louis Woerther, Antoine Martin, Elsa Poullot, Alain Wargnier, Jehane Fadlallah, Margaux Garzaro, Marion Malphettes, Claire Fieschi, Lucas Maisonobe, Hayat Bensekhri, Hélène Guillot, Rémi Bertinchamp, Marie Jachiet, Justine Poirot, Lionel Galicier, Eric Oksenhendler, David Boutboul |
Source: |
eJHaem, Vol 3, Iss 1, Pp 175-179 (2022) |
Publisher Information: |
Wiley, 2022. |
Publication Year: |
2022 |
Collection: |
LCC:Diseases of the blood and blood-forming organs |
Subject Terms: |
Castleman disease, chronic venous disease, lymphedema, Diseases of the blood and blood-forming organs, RC633-647.5 |
More Details: |
Abstract Idiopathic multicentric Castleman disease (iMCD) is a lymphoproliferative disease of unknown etiology. Deciphering mechanisms involved in CD pathogenesis may help improving patients’ care. Six cases of stereotyped sub‐diaphragmatic iMCD affecting lower limb‐draining areas and associated with severe and often ulcerative lower extremity chronic dermatological condition were identified in our cohort. Pathological examination revealed mixed or plasma‐cell type MCD. In three patients, shotgun metagenomics failed to identify any pathogen in involved lymph nodes. Antibiotics had a suspensive effect while rituximab and tocilizumab failed to improve the condition. This novel entity requires a specific approach and exclusion of potentially harmful immunomodulation. |
Document Type: |
article |
File Description: |
electronic resource |
Language: |
English |
ISSN: |
2688-6146 |
Relation: |
https://doaj.org/toc/2688-6146 |
DOI: |
10.1002/jha2.353 |
Access URL: |
https://doaj.org/article/b4f732957dcd4b52b76435837b644497 |
Accession Number: |
edsdoj.b4f732957dcd4b52b76435837b644497 |
Database: |
Directory of Open Access Journals |