Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies. (6th April 2022)

• Record Type: Journal Article
• Authors: Hernández-Molina, Gabriela; Kostov, Belchin; Brito-Zerón, Pilar; Vissink, Arjan; Mandl, Thomas; Hinrichs, Anneline C; Quartuccio, Luca; Baldini, Chiara; Seror, Raphaele; Szántó, Antonia; Isenberg, David; Gerli, Roberto; Nordmark, Gunnel; Rasmussen, Astrid; Solans-Laque, Roser; Hofauer, Benedikt; Sène, Damien; Pasoto, Sandra G; Rischmueller, Maureen; Praprotnik, Sonja; Gheita, Tamer A; Danda, Debashish; Armağan, Berkan; Suzuki, Yasunori; Valim, Valeria; Devauchelle-Pensec, Valerie; Retamozo, Soledad; Kvarnstrom, Marika; Sebastian, Agata; Atzeni, Fabiola; Giacomelli, Roberto; Carsons, Steven E; Kwok, Seung-Ki; Nakamura, Hideki; Fernandes Moça Trevisani, Virginia; Flores-Chávez, Alejandra; Mariette, Xavier; Ramos-Casals, Manuel;
• Is Part Of: Rheumatology. Volume 62:Number 1(2023)
• Page Start: 243
• Page End: 255
• Publication Date: 2022-04-06
• Subjects: SS -- haematological malignancy -- lymphoproliferative disease -- lymphoma -- MALT; Rheumatism -- Periodicals; Rheumatology -- Periodicals; 616.723005
• DOI: 10.1093/rheumatology/keac205
• Languages: English
• ISSNs: 1462-0324

Abstract

Abstract: Objective: To characterize 414 patients with primary SS who developed haematological malignancies and to analyse how the main SS- and lymphoma-related features can modify the presentation patterns and outcomes. Methods: By January 2021, the Big Data Sjögren Project Consortium database included 11 966 patients fulfilling the 2002/2016 classification criteria. Haematological malignancies diagnosed according to the World Health Organization (WHO) classification were retrospectively identified. Results: There were 414 patients (355 women, mean age 57 years) with haematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). A total of 376 (91%) patients had mature B-cell malignancy, nearly half had extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT lymphoma) ( n = 197), followed by diffuse large B-cell lymphoma (DLBCL) ( n = 67), nodal MZL lymphoma ( n = 29), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) ( n = 19) and follicular lymphoma (FL) ( n = 17). Rates of complete response, relapses and death were 80%, 34% and 13%, respectively, with a 5-year survival rate of 86.5% after a mean follow-up of 8 years. There were significant differences in age at diagnosis (younger in MALT, older in CLL/SLL), predominant clinical presentation (glandular enlargement in MALT lymphoma, peripheral lymphadenopathy in nodal MZL and FL, constitutional symptoms in DLBCL, incidental diagnosis in CLL/SLL), therapeutic response (higher in MALT lymphoma, lower in DLBCL) and survival (better in MALT, nodal MZL and FL, worse in DLBCL). Conclusion: In the largest reported study of haematological malignancies complicating primary SS, we confirm the overwhelming predominance of B-cell lymphomas, especially MALT, with the salivary glands being the primary site of involvement. This highly-specific histopathological scenario is linked with the overall good prognosis with a 5-year survival rate of nearly 90%.