Articles producció científica> Medicina i Cirurgia

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

  • Identification data

    Identifier: imarina:9443265
    Handle: https://hdl.handle.net/20.500.11797/imarina9443265
    Authors:
    Hernandez-Molina, GabrielaKostov, BelchinBrito-Zeron, PilarVissink, ArjanMandl, ThomasHinrichs, Anneline CQuartuccio, LucaBaldini, ChiaraSeror, RaphaeleSzanto, AntoniaIsenberg, DavidGerli, RobertoNordmark, GunnelRasmussen, AstridSolans-Laque, RoserHofauer, BenediktSene, DamienPasoto, Sandra GRischmueller, MaureenPraprotnik, SonjaGheita, Tamer ADanda, DebashishArmagan, BerkanSuzuki, YasunoriValim, ValeriaDevauchelle-Pensec, ValerieRetamozo, SoledadKvarnstrom, MarikaSebastian, AgataAtzeni, FabiolaGiacomelli, RobertoCarsons, Steven EKwok, Seung-KiNakamura, HidekiMoca Trevisani, Virginia FernandesFlores-Chavez, AlejandraMariette, XavierRamos-Casals, Manuel
    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 Sjogren 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 g

  • Others:

    Author, as appears in the article.: Hernandez-Molina, Gabriela; Kostov, Belchin; Brito-Zeron, Pilar; Vissink, Arjan; Mandl, Thomas; Hinrichs, Anneline C; Quartuccio, Luca; Baldini, Chiara; Seror, Raphaele; Szanto, Antonia; Isenberg, David; Gerli, Roberto; Nordmark, Gunnel; Rasmussen, Astrid; Solans-Laque, Roser; Hofauer, Benedikt; Sene, Damien; Pasoto, Sandra G; Rischmueller, Maureen; Praprotnik, Sonja; Gheita, Tamer A; Danda, Debashish; Armagan, 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; Moca Trevisani, Virginia Fernandes; Flores-Chavez, Alejandra; Mariette, Xavier; Ramos-Casals, Manuel
    Department: Medicina i Cirurgia
    URV's Author/s: López Dupla, Jesús Miguel
    Keywords: Ss Risk Prognosis Primary sjogrens-syndrome Predictors Non-hodgkin-lymphoma Mortalit Malt Mal Lymphoproliferative disease Lymphoma Health-organization classification Haematological malignancy Disease Biomarkers 2016 revision
    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 Sjogren 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%.
    Thematic Areas: Saúde coletiva Rheumatology Pharmacology (medical) Odontología Medicina iii Medicina ii Medicina i Interdisciplinar General medicine Farmacia Ensino Ciências biológicas iii Ciências biológicas ii Ciências biológicas i Ciência de alimentos Biotecnología
    licence for use: https://creativecommons.org/licenses/by/3.0/es/
    Author's mail: jesusmiguel.lopez@urv.cat
    Author identifier: 0000-0002-9141-2523
    Record's date: 2025-02-19
    Paper version: info:eu-repo/semantics/publishedVersion
    Paper original source: Rheumatology. 62 (1): 243-255
    APA: Hernandez-Molina, Gabriela; Kostov, Belchin; Brito-Zeron, Pilar; Vissink, Arjan; Mandl, Thomas; Hinrichs, Anneline C; Quartuccio, Luca; Baldini, Chiar (2023). Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies. Rheumatology, 62(1), 243-255. DOI: 10.1093/rheumatology/keac205
    Licence document URL: https://repositori.urv.cat/ca/proteccio-de-dades/
    Entity: Universitat Rovira i Virgili
    Journal publication year: 2023
    Publication Type: Journal Publications

  • Keywords:

    Pharmacology (Medical),Rheumatology
    Ss
    Risk
    Prognosis
    Primary sjogrens-syndrome
    Predictors
    Non-hodgkin-lymphoma
    Mortalit
    Malt
    Mal
    Lymphoproliferative disease
    Lymphoma
    Health-organization classification
    Haematological malignancy
    Disease
    Biomarkers
    2016 revision
    Saúde coletiva
    Rheumatology
    Pharmacology (medical)
    Odontología
    Medicina iii
    Medicina ii
    Medicina i
    Interdisciplinar
    General medicine
    Farmacia
    Ensino
    Ciências biológicas iii
    Ciências biológicas ii
    Ciências biológicas i
    Ciência de alimentos
    Biotecnología

  • Documents:

    DocumentPrincipal

  • Cerca a google

    Search to google scholar