Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies
Gabriela Hernández‐Molina, Belchin Kostov, Pilar Brito‐Zerón, Arjan Vissink, Thomas Mandl, Anneline C. Hinrichs, Luca Quartuccio, Chiara Baldini, Raphaèle Séror, Antónia Szántó, David Isenberg, Roberto Gerli, Gunnel Nordmark, Astrid Rasmussen, Roser Solans‐Laqué, Benedikt Hofauer, D. Sène, Sandra Gofinet Pasoto, Maureen Rischmueller, Sonja Praprotnik, Tamer A. Gheita, Debashish Danda, Berkan Armağan, Yasunori Suzuki, Valéria Valim, Valérie Devauchelle‐Pensec, Soledad Retamozo, Marika Kvarnström, Agata Sebastian, Fabiola Atzeni, Roberto Giacomelli, Steven E. Carsons, Seung‐Ki Kwok, Hideki Nakamura, Virgínia Fernandes Moça Trevisani, Alejandra Flores-Chávez, Xavier Mariette, Manuel Ramos‐Casals, Pilar Brito‐Zerón, Alejandra Flores-Chávez, Manuel Ramos‐Casals, Ildikó Fanny Horváth, Antónia Szántó, Tünde Tarr, F Ng, Astrid Rasmussen, Darise Farris, Dong Xu, Z Yan, Xingyuan Li, Baoshan Xu, Chiara Baldini, Stefano Bombardieri, Thomas Mandl, Peter Olsson, Roberta Priori, Federico Giardina, Raffaella Izzo, Raphaèle Séror, Xavier Mariette, Jacques‐Eric Gottenberg, Aike A. Kruize, Anneline C. Hinrichs, Hendrika Bootsma, Arjan Vissink, Debashish Danda, Pulukool Sandhya, Gabriela Hernández‐Molina, Jorge Sánchez‐Guerrero, Berkan Armağan, L Kilic, Umut Kalyoncu, Luca Quartuccio, Saviana Gandolfo, Salvatore De Vita, Sonja Praprotnik, Agata Sebastian, Piotr Wiland, Roberto Gerli, Elena Bartoloni, S-K Kwok, S-H Park, Marika Kvarnström, Marie Wahren‐Herlenius, Maureen Rischmueller, Sarah Downie‐Doyle, Roser Solans‐Laqué, D. Sène, Sandra Gofinet Pasoto, Yasunori Suzuki, M Kawano, David Isenberg, Gunnel Nordmark, Valéria Valim, Hideki Nakamura, Toshimasa Shimizu, S -Y Nishihata, Takashi Nakamura, Yukinori Takagi, Virgínia Fernandes Moça Trevisani
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%.