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Peer-reviewedCC BY 4.0Frontiers in Oncology

Characteristics of idiopathic multicentric Castleman disease in Spain: results of the registry-based ARCANA multicenter study

  • Jose-Tomas Navarro1
  • Santiago Montes-Moreno2
  • Grisell Starita Fajardo3
  • Manuel Espeso de Haro4
  • Pedro Durán-del-Campo5
  • Eva Gonzalez-Barca6
  • Antonio Gutiérrez7
  • Ana Jiménez-Ubieto8
  • Alejandro Martín García-Sancho9
  • Araceli Rubio-Martínez10
  • Rosario Sánchez-Martínez11
  • Paloma García-Martín12
6 more authors
  • Pablo Lorente-Alegre13
  • Iñigo Olazabal Eizaguirre14
  • Angel Ramirez Payer15
  • Guillermo Rodriguez16
  • Antonio García-Guiñon17
  • Andrés González-García18,19
19 affiliations
  1. 1Department of Hematology, Institut Català d’Oncologia. Josep Carreras Leukaemia Research Institute, Universitat Autònoma de Barcelona
  2. 2Department of Anatomic Pathology, Valdecilla/IDIVAL, Universidad de Cantabria
  3. 3Department of Internal Medicine, Hospital Universitario Ramón y Cajal
  4. 4Department Hematology and Hemotherapy, Hospital Regional Universitario de Málaga
  5. 5Department of Internal Medicine, Hospital Universitario Puerta de Hierro Majadahonda
  6. 6Department of Hematology, Institut Català D’Oncologia Hospitalet, IDIBELL, Universitat de Barcelona
  7. 7Department of Hematology, Son Espases University Hospital
  8. 8Department Haematology, Hospital 12 de Octubre
  9. 9Department Hematology, Hospital Universitario de Salamanca-IBSAL, CIC- IBMCC (USAL-CSIC), CIBERONC
  10. 10Department Hematology, University Hospital Miguel Servet
  11. 11Internal Medicine Department, Dr. Balmis General University Hospital, Alicante Institute for Health and Biomedical Research (ISABIAL)
  12. 12Department of Hematology and Hemotherapy, San Cecilio University Clinical Hospital
  13. 13Department of Hematology, Hospital Arnau de Vilanova-Lliria
  14. 14Department of Hematology, Hospital Universitario Donostia
  15. 15Department of Hematology, Hospital Universitario Central de Asturias
  16. 16Department of Hematology, Hospital Universitario Virgen del Rocío
  17. 17Department of Hematology, Hospital Arnau de Vilanova
  18. 18Systemic Autoinmmune Diseases Unit, Internal Medicine Department, Hospital Universitario Ramón y Cajal, IRYCIS
  19. 19Faculty of Medicine and Health Sciences, Universidad de Alcalá (UAH)
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Abstract

Objectives: We aimed to estimate the prevalence of iMCD in Spain by applying the current international consensus criteria.

Methods: This was an observational, multicenter, cross-sectional study that was conducted in 20 Spanish hospitals. The diagnosis of iMCD and the histological subtype were confirmed by a centralized scientific committee by applying the international consensus criteria. The local clinical, laboratory, and pathology reports were reviewed.

Results: Between February 2023 and April 2024, 30 patients with a documented diagnosis of iMCD were included in the study. The estimated prevalence of iMCD was 0·61 cases per 100,000 inhabitants (95% CI, 0·39–0·83); 23 subjects (76·7%) were classified as iMCD-NOS, and 7 (23·3%) were classified as iMCD-TAFRO. Twenty-two patients (73·3%) had received therapy for iMCD prior to their inclusion in the study. There are currently 21 patients (70%) on treatment, the most common being the use of siltuximab (19 patients; 90·4%). Complete response, partial response, and stable disease were reported in 11 (36·7%), 8 (26·7%), and 9 (30%) patients, respectively, with current treatment.

Conclusion: The prevalence of clinical cases of iMCD in Spain comparable to that reported in the literature. Siltuximab is currently the most commonly used therapy. Most patients under treatment showed a favorable clinical response.

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Cite

BibTeXRIS
BibTeX
@article{navarro2026characteristics,
  title = {{Characteristics of idiopathic multicentric Castleman disease in Spain: results of the registry-based ARCANA multicenter study}},
  author = {Jose-Tomas Navarro and Santiago Montes-Moreno and Grisell Starita Fajardo and Manuel Espeso de Haro and Pedro Durán-del-Campo and Eva Gonzalez-Barca and Antonio Gutiérrez and Ana Jiménez-Ubieto and Alejandro Martín García-Sancho and Araceli Rubio-Martínez and Rosario Sánchez-Martínez and Paloma García-Martín and Pablo Lorente-Alegre and Iñigo Olazabal Eizaguirre and Angel Ramirez Payer and Guillermo Rodriguez and Antonio García-Guiñon and Andrés González-García},
  journal = {Frontiers in Oncology},
  year = {2026},
  month = oct,
  doi = {10.3389/fonc.2026.1963499},
  url = {https://www.frontiersin.org/articles/10.3389/fonc.2026.1963499/full},
  note = {CC BY 4.0},
}
RIS
TY  - JOUR
TI  - Characteristics of idiopathic multicentric Castleman disease in Spain: results of the registry-based ARCANA multicenter study
AU  - Jose-Tomas Navarro
AU  - Santiago Montes-Moreno
AU  - Grisell Starita Fajardo
AU  - Manuel Espeso de Haro
AU  - Pedro Durán-del-Campo
AU  - Eva Gonzalez-Barca
AU  - Antonio Gutiérrez
AU  - Ana Jiménez-Ubieto
AU  - Alejandro Martín García-Sancho
AU  - Araceli Rubio-Martínez
AU  - Rosario Sánchez-Martínez
AU  - Paloma García-Martín
AU  - Pablo Lorente-Alegre
AU  - Iñigo Olazabal Eizaguirre
AU  - Angel Ramirez Payer
AU  - Guillermo Rodriguez
AU  - Antonio García-Guiñon
AU  - Andrés González-García
PY  - 2026
DA  - 2026/10/19
JO  - Frontiers in Oncology
DO  - 10.3389/fonc.2026.1963499
UR  - https://www.frontiersin.org/articles/10.3389/fonc.2026.1963499/full
AB  - Objectives: We aimed to estimate the prevalence of iMCD in Spain by applying the current international consensus criteria.  Methods: This was an observational, multicenter, cross-sectional study that was conducted in 20 Spanish hospitals. The diagnosis of iMCD and the histological subtype were confirmed by a centralized scientific committee by applying the international consensus criteria. The local clinical, laboratory, and pathology reports were reviewed.  Results: Between February 2023 and April 2024, 30 patients with a documented diagnosis of iMCD were included in the study. The estimated prevalence of iMCD was 0·61 cases per 100,000 inhabitants (95% CI, 0·39–0·83); 23 subjects (76·7%) were classified as iMCD-NOS, and 7 (23·3%) were classified as iMCD-TAFRO. Twenty-two patients (73·3%) had received therapy for iMCD prior to their inclusion in the study. There are currently 21 patients (70%) on treatment, the most common being the use of siltuximab (19 patients; 90·4%). Complete response, partial response, and stable disease were reported in 11 (36·7%), 8 (26·7%), and 9 (30%) patients, respectively, with current treatment.  Conclusion: The prevalence of clinical cases of iMCD in Spain comparable to that reported in the literature. Siltuximab is currently the most commonly used therapy. Most patients under treatment showed a favorable clinical response.
SN  - 2234-943X
ER  -