ENFERMEDAD DE CASTLEMAN MULTICÉNTRICA: DE LO COMÚN A LO INUSUAL. CASO CLÍNICO.

Justina Bustos de Chinchilla; Onica Pinzón; José Garrido

Submitted November 15, 2024

Published 2026-02-02

Artículos de Investigación

Vol. 35 No. 1 (2022): Revista Médico Científica

MULTICENTRIC CASTLEMAN'S DISEASE: FROM THE COMMON TO THE UNUSUAL. CLINICAL CASE.

Justina Bustos de Chinchilla⁺⁻
Onica Pinzón⁺⁻
José Garrido⁺⁻

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DOI: ND

Published: 2026-02-02

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Abstract

We present the case of a 49-year-old patient who debuted with B symptoms, neurological involvement, diffuse lymphadenopathy, and autoimmune hemolytic anemia due to warm antibodies. With a wide differential diagnosis, an excisional biopsy of the axillary lymph nodes was performed, reaching the histological diagnosis of Multicenter Castleman's disease with the presence of DNA from Hhuman Hherpesvirus--8 in plasma determined by quantitative polymerase chain reaction (PCR). The patient was managed with chemotherapy composed of rituximab, liposomal doxorubicin, cyclophosphamide, vincristinevincristine, and dexamethasone with excellent therapeutic response.

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References

Cervantes C, Correa R. Castleman Disease: A Rare Condition with Endocrine Manifestations. Cureus. 2015;7(11):e380–e380.
Munshi N, Mehra M, van de Velde H, Desai A, Potluri R, Vermeulen J. Use of a claims database to characterize and estimate the incidence rate for Castleman disease. Leuk Lymphoma. 2015;56(5):1252–1260.
Fajgenbaum D. Unicentric CastlemanDisease. En: Freedman A, Connor R, editores. UpToDate (consultado 30 de enero 2021). Disponible en: https://www.uptodate.com/contents/unicentric-castleman-disease#H3029239
Fajgenbaum D, Uldrick T, Bagg A. International, evidence-based consensus diagnostic criteria for HHV-8–negative/idiopathic multicentric Castleman disease. Blood. 2017;129(12):1646–1657.
Wu D, Lim M, Jaffe E. Pathology of Castleman Disease. Hematol Oncol Clin North Am. 2018;32(1):37?52.
Fajgenbaum DC, van Rhee F, Nabel CS. HHV-8-negative, idiopathic multicentric Castlemandisease: novel insights into biology, pathogenesis, and therapy. Blood. 2014;123(19), 2924–2933.
Li P, Liu H, Li H, Li A, Yu G, Yin W. Hyaline vascular variant of unicentric Castleman disease of the tonsil: a case report. Diagn Pathol. 2019;14(1):70.
Fajgenbaum DC, Shilling D. Castleman disease pathogenesis. Hematol Oncol Clin North Am. 2018;32(1):11-21.
Soumerai JD, Sohani AR, Abramson JS. Diagnosis and management of Castleman disease. Cancer Control. 2014;21(4):266?278.
Kapriniotis K, Lampridis S, Mitsos S, Patrini D, Lawrence DR, Panagiotopoulos N. Biologic Agents in the Treatment of Multicentric Castleman Disease. Turk Thorac J. 2018;19(4):220-225.
Borie R, Wislez M, Antoine M, Cadranel J. Lymphoproliferative Disorders of the Lung. Respiration. 2017;94(2):157-175
Wojty? M, Piekarska A, Kunc M, Ptaszynski K, Biernat W, Zaucha JM, et al. Clinicopathological comparison and therapeutic approach to Castleman disease-a case-based review. J Thorac Dis. 2019;11(11):4859?4874.
Alzahrani MF, Radwi M, Leitch HA. Practical Management of Castleman's Disease. Acta Haematol. 2016;136(1):16?22.
van Rhee F, Voorhees P, Dispenzieri A, Fossa A, Srkalovic G, Ide M, et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castlemandisease. Blood. 2018;132(20), 2115–2124.
Chan KL, Lade S, Prince HM, Harrison SJ. Update and new approaches in the treatment of Castleman disease. J Blood Med. 2016;7:145-58.

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[1] Cervantes C, Correa R. Castleman Disease: A Rare Condition with Endocrine Manifestations. Cureus. 2015;7(11):e380–e380.

[2] Munshi N, Mehra M, van de Velde H, Desai A, Potluri R, Vermeulen J. Use of a claims database to characterize and estimate the incidence rate for Castleman disease. Leuk Lymphoma. 2015;56(5):1252–1260.

[3] Fajgenbaum D. Unicentric CastlemanDisease. En: Freedman A, Connor R, editores. UpToDate (consultado 30 de enero 2021). Disponible en: https://www.uptodate.com/contents/unicentric-castleman-disease#H3029239

[4] Fajgenbaum D, Uldrick T, Bagg A. International, evidence-based consensus diagnostic criteria for HHV-8–negative/idiopathic multicentric Castleman disease. Blood. 2017;129(12):1646–1657.

[5] Wu D, Lim M, Jaffe E. Pathology of Castleman Disease. Hematol Oncol Clin North Am. 2018;32(1):37?52.

[6] Fajgenbaum DC, van Rhee F, Nabel CS. HHV-8-negative, idiopathic multicentric Castlemandisease: novel insights into biology, pathogenesis, and therapy. Blood. 2014;123(19), 2924–2933.

[7] Li P, Liu H, Li H, Li A, Yu G, Yin W. Hyaline vascular variant of unicentric Castleman disease of the tonsil: a case report. Diagn Pathol. 2019;14(1):70.

[8] Fajgenbaum DC, Shilling D. Castleman disease pathogenesis. Hematol Oncol Clin North Am. 2018;32(1):11-21.

[9] Soumerai JD, Sohani AR, Abramson JS. Diagnosis and management of Castleman disease. Cancer Control. 2014;21(4):266?278.

[10] Kapriniotis K, Lampridis S, Mitsos S, Patrini D, Lawrence DR, Panagiotopoulos N. Biologic Agents in the Treatment of Multicentric Castleman Disease. Turk Thorac J. 2018;19(4):220-225.

[11] Borie R, Wislez M, Antoine M, Cadranel J. Lymphoproliferative Disorders of the Lung. Respiration. 2017;94(2):157-175

[12] Wojty? M, Piekarska A, Kunc M, Ptaszynski K, Biernat W, Zaucha JM, et al. Clinicopathological comparison and therapeutic approach to Castleman disease-a case-based review. J Thorac Dis. 2019;11(11):4859?4874.

[13] Alzahrani MF, Radwi M, Leitch HA. Practical Management of Castleman's Disease. Acta Haematol. 2016;136(1):16?22.

[14] van Rhee F, Voorhees P, Dispenzieri A, Fossa A, Srkalovic G, Ide M, et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castlemandisease. Blood. 2018;132(20), 2115–2124.

[15] Chan KL, Lade S, Prince HM, Harrison SJ. Update and new approaches in the treatment of Castleman disease. J Blood Med. 2016;7:145-58.

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