DOI: 10.12890/2026_006166
Concurrent idiopathic multicentric Castleman disease with TAFRO and Sjögren’s syndrome in a young patient: a challenging diagnostic overlap
Authors
mariacarolinalcarvalho@gmail.com
Keywords
Multicentric Castleman disease, TAFRO syndrome, Sjögren’s syndrome, hyperinflammationAbstract
Background: Idiopathic multicentric Castleman disease (iMCD) with TAFRO syndrome (characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, organomegaly) is a recently described disorder. The diagnostic criteria for iMCD-TAFRO exclude patients with concomitant autoimmune diseases such as primary Sjögren’s syndrome. However, recent case reports suggest that although rare, these conditions may coexist.
Case report: We report the case of a 37-year-old woman who presented with escalating systemic inflammation, cytopenias, anasarca, renal dysfunction and organomegaly, with lymph node biopsy suggestive of Castleman disease. Simultaneously, dense lacrimal gland hyperplasia with histopathology demonstrating lymphoepithelial sialadenitis and strongly positive anti-Ro52 antibodies led to a diagnosis of Sjögren’s syndrome. Given the rapid clinical deterioration and marked hyperinflammation, corticosteroid therapy and tocilizumab were implemented, leading to sustained remission.
Conclusion: This case illustrates the diagnostic complexity posed by overlapping features of Idiopathic multicentric Castleman disease with TAFRO syndrome and Sjögren’s syndrome and reinforces the need to reconsider existing diagnostic criteria to ensure timely diagnosis and effective treatment. Epidemiologically, this is also a rare case of iMCD with TAFRO syndrome in a patient of African descent, contrasting with previous reports predominantly involving Asian individuals.
References
- Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood 2020;135:1353-1364. doi: 10.1182/blood.2019000931
- Dirnhofer S, Nishimura Y, Dispenzieri A. Non-Clonal Lymphoproliferative Diseases. Hematol Oncol 2025;43:e70065. doi: 10.1002/hon.70065
- Nishimura Y, Fajgenbaum DC, Pierson SK, Iwaki N, Nishikori A, Kawano M, et al. Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease. Am J Hematol 2021;96:1241-1252. doi: 10.1002/ajh.26292
- Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood 2017;129:1646-1657. doi: 10.1182/blood-2016-10-746933
- Kakutani T, Kamada R, Tamai Y. Pathophysiology, Treatment, and Prognosis of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome: A Review. Curr Issues Mol Biol 2024;46:11255-11269. doi: 10.3390/cimb46100668
- Li ZY, Kim S, Huang S, Mian R. Multicentric Castleman disease with TAFRO syndrome and Sjögren’s. Clin Case Rep 2019;7:2388-2392. doi: 10.1002/ccr3.2502
- Tromby F, Manfrè V, Chatzis LG, Arends S, Tzioufas AG, Bootsma H, et al. Clinical manifestations, imaging and treatment of Sjögren’s disease: one year in review 2024. Clin Exp Rheumatol 2024;42:2322-2335. doi: 10.55563/clinexprheumatol/5xq3fb
- Grange L, Chalayer E, Boutboul D, Paul S, Galicier L, Gramont B, et al. TAFRO syndrome: A severe manifestation of Sjögren’s syndrome? A systematic review. Autoimmun Rev 2022;21:103137. doi: 10.1016/j.autrev.2022.103137
- Van Rhee F, Voorhees P, Dispenzieri A, Fossa A, Srkalovi G, Ide M, et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood 2018;132:2115-2124. doi: 10.1182/blood-2018-07-862334
- Nashimoto N, Kanakura Y, Aozasa K, Johkoh T, Nakamura M, Nakano S, et al. Humanized anti-interleukin-6 receptor antibody treatment of multicentric Castleman disease. Blood 2005;106:2627-2632. doi: 10.1182/blood-2004-12-4602
