Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies

Hern('a)ndez-Molina, G.; Kostov, B.; Brito-Zer('o)n, P.; Vissink, A.; Mandl, T.; Hinrichs, A. C.; Quartuccio, L.; Baldini, C.; Seror, R.; Sz('a)nt('o), A.; Isenberg, D.; Gerli, R.; Nordmark, G.; Rasmussen, A.; Solans-Laque, R.; Hofauer, B.; S(`e)ne, D.; Pasoto, S. G.; Rischmueller, M.; Praprotnik, S.; Gheita, T. A.; Danda, D.; Arma?an, B.; Suzuki, Y.; Valim, V.; Devauchelle-Pensec, V.; Retamozo, S.; Kvarnstrom, M.; Sebastian, A.; Atzeni, F.; Giacomelli, R.; Carsons, S. E.; Kwok, S. -K.; Nakamura, H.; Fernandes Mo?a Trevisani, V.; Flores-Ch('a)vez, A.; Mariette, X.; Ramos-Casals, M.; Horvath, I. F.; Tarr, T.; Ng, F.; Farris, D. A.; Dong, X.; Yan, Z.; Li, X.; Xu, B.; Bombardieri, S.; Olsson, P.; Priori, R.; Giardina, F.; Izzo, R.; Gottenberg, J. E.; Kruize, A. A.; Hinrichs, A.; Bootsma, H.; Sandhya, P.; Hernandez-Molina, G.; S('a)nchez-Guerrero, J.; Armagan, B.; Kilic, L.; Kalyoncu, U.; Gandolfo, S.; De Vita, S.; Wiland, P.; Bartoloni, E.; Park, S. -H.; Wahren-Herlenius, M.; Downie-Doyle, S.; Sene, D.; Kawano, M.; Shimizu, T.; Nishihata, S. -Y.; Nakamura, T.; Takagi, Y.; Knopf, A.; Fraile, G.; Saraux, A.; Bombardieri, M.; Astorri, E.; Hammenfors, D.; Brun, J. G.; Maure Noia, B.; Argibay Filgueira, A. B.; S('a)nchez Bern('a), I.; L('o)pez Dupla, M.; Alberto Rojas, R.; Febrer Nafria, A. M.; Morel, J.; Fonseca Aizpuru, E.; Santos Seoane, S.; Morcillo, C.; Melchor D('i)az, S.; Carreira, P.; Vollenveider, C.; V('a)zquez, M.; Ericka D('i)az Cuiza, P.; Herrera, B. E.; Andrea Consani, S.; Comotto, A.; De Miguel Campo, B.; Sis('o)-Almirall, A.; Acar-Denizli, N.

doi:10.1093/rheumatology/keac205

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%.

Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies / Hern('a)ndez-Molina, G., Kostov, B., Brito-Zer('o)n, P., Vissink, A., Mandl, T., Hinrichs, A.C., Quartuccio, L., Baldini, C., Seror, R., Sz('a)nt('o), A., Isenberg, D., Gerli, R., Nordmark, G., Rasmussen, A., Solans-Laque, R., Hofauer, B., S(`e)ne, D., Pasoto, S.G., Rischmueller, M., Praprotnik, S., et al.. - In: RHEUMATOLOGY. - ISSN 1462-0332. - 62:1(2023), pp. 243-255. [10.1093/rheumatology/keac205]