Leg‐type form of idiopathic multicentric Castleman disease associated with severe lower extremity chronic venous/lymphatic disease

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...

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Published inEJHaem Vol. 3; no. 1; pp. 175 - 179
Main Authors Ballul, Thomas, Belfeki, Nabil, Masson, Adèle, Meignin, Véronique, Woerther, Paul‐Louis, Martin, Antoine, Poullot, Elsa, Wargnier, Alain, Fadlallah, Jehane, Garzaro, Margaux, Malphettes, Marion, Fieschi, Claire, Maisonobe, Lucas, Bensekhri, Hayat, Guillot, Hélène, Bertinchamp, Rémi, Jachiet, Marie, Poirot, Justine, Galicier, Lionel, Oksenhendler, Eric, Boutboul, David
Format Journal Article
LanguageEnglish
Published Hoboken John Wiley & Sons, Inc 01.02.2022
Wiley
John Wiley and Sons Inc
Subjects
Antibiotics
Antibodies
Antigens
Biopsy
Castleman disease
Castleman's disease
chronic venous disease
Congenital diseases
Diabetes
Etiology
HIV
Human immunodeficiency virus
Immunomodulation
Immunoproliferative diseases
Leg ulcers
Life Sciences
Lymph nodes
Lymphatic diseases
Lymphatic system
Lymphedema
Lymphocytes
Metabolism
Metagenomics
Obesity
Pathology
Patients
Plasma
Remission (Medicine)
Rituximab
Short Report
Short Reports
Tomography
Tuberculosis
Vascular endothelial growth factor
Weight control
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Summary: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.
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ISSN:2688-6146
2688-6146
DOI:10.1002/jha2.353