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

Thomas Ballul; Nabil Belfeki; Adèle de Masson; 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

doi:10.1002/jha2.353

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

EJHaem. 2021 Dec 23;3(1):175-179. doi: 10.1002/jha2.353. eCollection 2022 Feb.

Authors

Thomas Ballul¹, Nabil Belfeki², Adèle de Masson³, Véronique Meignin⁴, Paul-Louis Woerther⁵, Antoine Martin⁶, Elsa Poullot⁷, Alain Wargnier⁸, Jehane Fadlallah^{1

9}, Margaux Garzaro^{1

9}, Marion Malphettes^{1

9}, Claire Fieschi^{1

9}, Lucas Maisonobe¹, Hayat Bensekhri¹⁰, Hélène Guillot¹¹, Rémi Bertinchamp^{1

9}, Marie Jachiet³, Justine Poirot¹², Lionel Galicier^{1

9}, Eric Oksenhendler^{1

9}, David Boutboul^{1

9

12}

Affiliations

¹ Clinical Immunology Department Hôpital Saint Louis, Université de Paris Paris France.
² Internal Medicine Department Centre hospitalier de Melun Melun France.
³ Dermatology Department Université de Paris Paris France.
⁴ Pathology Department Hôpital Saint Louis Université de Paris Paris France.
⁵ Bacteriology and Virology Department Hôpital Henri Mondor Créteil France.
⁶ Pathology Department Hôpital Avicenne Université Sorbonne Paris Nord Bobigny France.
⁷ Pathology Department Hôpital Henri Mondor Université Paris-Est Créteil Créteil France.
⁸ Bacteriology Department Hôpital Saint Louis Université de Paris Paris France.
⁹ National Reference Center for Castleman disease Hôpital Saint Louis Université de Paris Paris France.
¹⁰ Internal Medicine Department Groupe Hospitalier Nord Essonne Longjumeau France.
¹¹ Internal Medicine Department Hôpital Robert Ballanger Université Sorbonne Paris Nord Aulnay-Sous-Bois France.
¹² INSERM U976 HIPI, Hôpital Saint Louis Université de Paris Paris France.

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.

Keywords: Castleman disease; chronic venous disease; lymphedema.