dc.contributor.author | Hernández-Molina, G | en_US |
dc.contributor.author | Kostov, B | en_US |
dc.contributor.author | Brito-Zerón, P | en_US |
dc.contributor.author | Vissink, A | en_US |
dc.contributor.author | Mandl, T | en_US |
dc.contributor.author | Hinrichs, AC | en_US |
dc.contributor.author | Quartuccio, L | en_US |
dc.contributor.author | Baldini, C | en_US |
dc.contributor.author | Seror, R | en_US |
dc.contributor.author | Szántó, A | en_US |
dc.contributor.author | Isenberg, D | en_US |
dc.contributor.author | Gerli, R | en_US |
dc.contributor.author | Nordmark, G | en_US |
dc.contributor.author | Rasmussen, A | en_US |
dc.contributor.author | Solans-Laque, R | en_US |
dc.contributor.author | Hofauer, B | en_US |
dc.contributor.author | Sène, D | en_US |
dc.contributor.author | Pasoto, SG | en_US |
dc.contributor.author | Rischmueller, M | en_US |
dc.contributor.author | Praprotnik, S | en_US |
dc.contributor.author | Gheita, TA | en_US |
dc.contributor.author | Danda, D | en_US |
dc.contributor.author | Armağan, B | en_US |
dc.contributor.author | Suzuki, Y | en_US |
dc.contributor.author | Valim, V | en_US |
dc.contributor.author | Devauchelle-Pensec, V | en_US |
dc.contributor.author | Retamozo, S | en_US |
dc.contributor.author | Kvarnstrom, M | en_US |
dc.contributor.author | Sebastian, A | en_US |
dc.contributor.author | Atzeni, F | en_US |
dc.contributor.author | Giacomelli, R | en_US |
dc.contributor.author | Carsons, SE | en_US |
dc.contributor.author | Kwok, S-K | en_US |
dc.contributor.author | Nakamura, H | en_US |
dc.contributor.author | Fernandes Moça Trevisani, V | en_US |
dc.contributor.author | Flores-Chávez, A | en_US |
dc.contributor.author | Mariette, X | en_US |
dc.contributor.author | Ramos-Casals, M | en_US |
dc.contributor.author | Sjögren Big Data Consortium | en_US |
dc.date.accessioned | 2023-11-30T10:36:52Z | |
dc.date.available | 2023-11-30T10:36:52Z | |
dc.date.issued | 2022-12-23 | en_US |
dc.identifier.uri | https://qmro.qmul.ac.uk/xmlui/handle/123456789/92480 | |
dc.description.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%. | en_US |
dc.format.extent | 243 - 255 | en_US |
dc.language | eng | en_US |
dc.relation.ispartof | Rheumatology (Oxford) | en_US |
dc.subject | MALT | en_US |
dc.subject | SS | en_US |
dc.subject | haematological malignancy | en_US |
dc.subject | lymphoma | en_US |
dc.subject | lymphoproliferative disease | en_US |
dc.subject | Humans | en_US |
dc.subject | Female | en_US |
dc.subject | Middle Aged | en_US |
dc.subject | Lymphoma, B-Cell, Marginal Zone | en_US |
dc.subject | Leukemia, Lymphocytic, Chronic, B-Cell | en_US |
dc.subject | Retrospective Studies | en_US |
dc.subject | Lymphoma, Follicular | en_US |
dc.subject | Lymphoma, Large B-Cell, Diffuse | en_US |
dc.subject | Hematologic Neoplasms | en_US |
dc.subject | World Health Organization | en_US |
dc.title | Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies. | en_US |
dc.type | Article | |
dc.identifier.doi | 10.1093/rheumatology/keac205 | en_US |
pubs.author-url | https://www.ncbi.nlm.nih.gov/pubmed/35385104 | en_US |
pubs.issue | 1 | en_US |
pubs.notes | Not known | en_US |
pubs.publication-status | Published | en_US |
pubs.volume | 62 | en_US |