Leg‐type form of idiopathic multicentric Castleman disease associated with severe lower extremity chronic venous/lymphatic disease - Université Paris-Est-Créteil-Val-de-Marne Access content directly
Journal Articles eJHaem Year : 2021

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

Thomas Ballul
  • Function : Author
Nabil Belfeki
  • Function : Author
Adèle de Masson
  • Function : Author
Véronique Meignin
  • Function : Author
Antoine Martin
  • Function : Author
Elsa Poullot
  • Function : Author
Alain Wargnier
  • Function : Author
Jehane Fadlallah
  • Function : Author
Margaux Garzaro
  • Function : Author
Marion Malphettes
Claire Fieschi
  • Function : Author
Lucas Maisonobe
  • Function : Author
Hayat Bensekhri
  • Function : Author
Hélène Guillot
  • Function : Author
Rémi Bertinchamp
  • Function : Author
Marie Jachiet
  • Function : Author
Justine Poirot
  • Function : Author
Lionel Galicier
  • Function : Author
Eric Oksenhendler
David Boutboul

Abstract

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.

Dates and versions

hal-04298101 , version 1 (21-11-2023)

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Cite

Thomas Ballul, Nabil Belfeki, Adèle de Masson, Véronique Meignin, Paul‐louis Woerther, et al.. Leg‐type form of idiopathic multicentric Castleman disease associated with severe lower extremity chronic venous/lymphatic disease. eJHaem, 2021, 3 (1), pp.175-179. ⟨10.1002/jha2.353⟩. ⟨hal-04298101⟩
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