TY - JOUR
T1 - Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies
AU - the Sjögren Big Data Consortium
AU - Hernández-Molina, Gabriela
AU - Kostov, Belchin
AU - Brito-Zerón, Pilar
AU - Vissink, Arjan
AU - Mandl, Thomas
AU - Hinrichs, Anneline C.
AU - Quartuccio, Luca
AU - Baldini, Chiara
AU - Seror, Raphaele
AU - Szántó, Antonia
AU - Isenberg, David
AU - Gerli, Roberto
AU - Nordmark, Gunnel
AU - Rasmussen, Astrid
AU - Solans-Laque, Roser
AU - Hofauer, Benedikt
AU - Sène, Damien
AU - Pasoto, Sandra G.
AU - Rischmueller, Maureen
AU - Praprotnik, Sonja
AU - Gheita, Tamer A.
AU - Danda, Debashish
AU - Armaǧan, Berkan
AU - Suzuki, Yasunori
AU - Valim, Valeria
AU - Devauchelle-Pensec, Valerie
AU - Retamozo, Soledad
AU - Kvarnstrom, Marika
AU - Sebastian, Agata
AU - Atzeni, Fabiola
AU - Giacomelli, Roberto
AU - Carsons, Steven E.
AU - Kwok, Seung Ki
AU - Nakamura, Hideki
AU - Fernandes Moça Trevisani, Virginia
AU - Flores-Chávez, Alejandra
AU - Mariette, Xavier
AU - Ramos-Casals, Manuel
AU - Horvath, I. F.
AU - Tarr, T.
AU - Ng, F.
AU - Farris, D. A.
AU - Dong, X.
AU - Yan, Z.
AU - Li, X.
AU - Xu, B.
AU - Bombardieri, S.
AU - Olsson, P.
AU - Priori, R.
AU - Giardina, F.
N1 - Publisher Copyright:
© 2022 The Author(s). Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved.
PY - 2023/1/1
Y1 - 2023/1/1
N2 - 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%.
AB - 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%.
KW - MALT
KW - SS
KW - haematological malignancy
KW - lymphoma
KW - lymphoproliferative disease
UR - https://www.scopus.com/pages/publications/85139097101
U2 - 10.1093/rheumatology/keac205
DO - 10.1093/rheumatology/keac205
M3 - Article
C2 - 35385104
AN - SCOPUS:85139097101
SN - 1462-0324
VL - 62
SP - 243
EP - 255
JO - Rheumatology
JF - Rheumatology
IS - 1
ER -