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

Gabriela Hernández-Molina; Belchin Kostov; Pilar Brito-Zerón; Arjan Vissink; Thomas Mandl; Anneline C Hinrichs; Luca Quartuccio; Chiara Baldini; Raphaele Seror; Antonia Szántó; David Isenberg; Roberto Gerli; Gunnel Nordmark; Astrid Rasmussen; Roser Solans-Laque; Benedikt Hofauer; Damien Sène; Sandra G Pasoto; Maureen Rischmueller; Sonja Praprotnik; Tamer A Gheita; Debashish Danda; Berkan Armağan; Yasunori Suzuki; Valeria Valim; Valerie Devauchelle-Pensec; Soledad Retamozo; Marika Kvarnstrom; Agata Sebastian; Fabiola Atzeni; Roberto Giacomelli; Steven E Carsons; Seung-Ki Kwok; Hideki Nakamura; Virginia Fernandes Moça Trevisani; Alejandra Flores-Chávez; Xavier Mariette; Manuel Ramos-Casals; Sjögren Big Data Consortium

doi:10.1093/rheumatology/keac205

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

Gabriela Hernández-Molina, Belchin Kostov, Pilar Brito-Zerón, Arjan Vissink, Thomas Mandl, Anneline C Hinrichs, Luca Quartuccio, Chiara Baldini, Raphaele Seror, Antonia Szántó, David Isenberg, Roberto Gerli, Gunnel Nordmark, Astrid Rasmussen, Roser Solans-Laque, Benedikt Hofauer, Damien Sène, Sandra G Pasoto, Maureen Rischmueller, Sonja PraprotnikTamer A Gheita, Debashish Danda, Berkan Armağan, Yasunori Suzuki, Valeria Valim, Valerie Devauchelle-Pensec, Soledad Retamozo, Marika Kvarnstrom, Agata Sebastian, Fabiola Atzeni, Roberto Giacomelli, Steven E Carsons, Seung-Ki Kwok, Hideki Nakamura, Virginia Fernandes Moça Trevisani, Alejandra Flores-Chávez, Xavier Mariette, Manuel Ramos-Casals, Sjögren Big Data Consortium

Rheumatology

Research output: Contribution to journal › Article › peer-review

Abstract

OBJECTIVE: To characterize 414 patients with primary SS who developed hematological malignancies and to analyze 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. Hematological 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 hematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). 376 (91%) patients had mature B cell malignancy, nearly half MALT lymphoma (n = 197), followed by DLBCL (n = 67), nodal MZL lymphoma (n = 29), CLL/SLL (n = 19) and follicular lymphoma (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 hematological 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%.

Original language	English
Pages (from-to)	243-255
Journal	Rheumatology (Oxford, England)
Volume	62
Issue number	1
Early online date	2022
DOIs	https://doi.org/10.1093/rheumatology/keac205
Publication status	Published - 2022

Subject classification (UKÄ)

Cancer and Oncology
Rheumatology and Autoimmunity
Hematology

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1093/rheumatology/keac205

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Hernández-Molina, G., Kostov, B., Brito-Zerón, P., Vissink, A., Mandl, T., Hinrichs, A. C., Quartuccio, L., Baldini, C., Seror, R., Szántó, A., Isenberg, D., Gerli, R., Nordmark, G., Rasmussen, A., Solans-Laque, R., Hofauer, B., Sène, D., Pasoto, S. G., Rischmueller, M., ... Sjögren Big Data Consortium (2022). Characterization and outcomes of 414 patients with primary SS who developed hematological malignancies. Rheumatology (Oxford, England), 62(1 ), 243-255. https://doi.org/10.1093/rheumatology/keac205

@article{f9c4b5dfb8b74b1eb93978b82885df85,

title = "Characterization and outcomes of 414 patients with primary SS who developed hematological malignancies",

abstract = "OBJECTIVE: To characterize 414 patients with primary SS who developed hematological malignancies and to analyze how the main SS- and lymphoma-related features can modify the presentation patterns and outcomes.METHODS: By January 2021, the Big Data Sj{\"o}gren Project Consortium database included 11 966 patients fulfilling the 2002/2016 classification criteria. Hematological 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 hematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). 376 (91%) patients had mature B cell malignancy, nearly half MALT lymphoma (n = 197), followed by DLBCL (n = 67), nodal MZL lymphoma (n = 29), CLL/SLL (n = 19) and follicular lymphoma (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 hematological 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%.",

author = "Gabriela Hern{\'a}ndez-Molina and Belchin Kostov and Pilar Brito-Zer{\'o}n and Arjan Vissink and Thomas Mandl and Hinrichs, {Anneline C} and Luca Quartuccio and Chiara Baldini and Raphaele Seror and Antonia Sz{\'a}nt{\'o} and David Isenberg and Roberto Gerli and Gunnel Nordmark and Astrid Rasmussen and Roser Solans-Laque and Benedikt Hofauer and Damien S{\`e}ne and Pasoto, {Sandra G} and Maureen Rischmueller and Sonja Praprotnik and Gheita, {Tamer A} and Debashish Danda and Berkan Armağan and Yasunori Suzuki and Valeria Valim and Valerie Devauchelle-Pensec and Soledad Retamozo and Marika Kvarnstrom and Agata Sebastian and Fabiola Atzeni and Roberto Giacomelli and Carsons, {Steven E} and Seung-Ki Kwok and Hideki Nakamura and {Fernandes Mo{\c c}a Trevisani}, Virginia and Alejandra Flores-Ch{\'a}vez and Xavier Mariette and Manuel Ramos-Casals and {Sj{\"o}gren Big Data Consortium}",

note = "{\textcopyright} The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: [email protected].",

year = "2022",

doi = "10.1093/rheumatology/keac205",

language = "English",

volume = "62",

pages = "243--255",

journal = "Rheumatology (Oxford, England)",

issn = "1462-0332",

publisher = "Oxford University Press",

number = "1 ",

}

Hernández-Molina, G, Kostov, B, Brito-Zerón, P, Vissink, A, Mandl, T, Hinrichs, AC, Quartuccio, L, Baldini, C, Seror, R, Szántó, A, Isenberg, D, Gerli, R, Nordmark, G, Rasmussen, A, Solans-Laque, R, Hofauer, B, Sène, D, Pasoto, SG, Rischmueller, M, Praprotnik, S, Gheita, TA, Danda, D, Armağan, B, Suzuki, Y, Valim, V, Devauchelle-Pensec, V, Retamozo, S, Kvarnstrom, M, Sebastian, A, Atzeni, F, Giacomelli, R, Carsons, SE, Kwok, S-K, Nakamura, H, Fernandes Moça Trevisani, V, Flores-Chávez, A, Mariette, X, Ramos-Casals, M & Sjögren Big Data Consortium 2022, 'Characterization and outcomes of 414 patients with primary SS who developed hematological malignancies', Rheumatology (Oxford, England), vol. 62, no. 1 , pp. 243-255. https://doi.org/10.1093/rheumatology/keac205

TY - JOUR

T1 - Characterization and outcomes of 414 patients with primary SS who developed hematological malignancies

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 - Armağan, 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 - Sjögren Big Data Consortium

PY - 2022

Y1 - 2022

N2 - OBJECTIVE: To characterize 414 patients with primary SS who developed hematological malignancies and to analyze 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. Hematological 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 hematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). 376 (91%) patients had mature B cell malignancy, nearly half MALT lymphoma (n = 197), followed by DLBCL (n = 67), nodal MZL lymphoma (n = 29), CLL/SLL (n = 19) and follicular lymphoma (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 hematological 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 hematological malignancies and to analyze 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. Hematological 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 hematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). 376 (91%) patients had mature B cell malignancy, nearly half MALT lymphoma (n = 197), followed by DLBCL (n = 67), nodal MZL lymphoma (n = 29), CLL/SLL (n = 19) and follicular lymphoma (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 hematological 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%.

U2 - 10.1093/rheumatology/keac205

DO - 10.1093/rheumatology/keac205

M3 - Article

C2 - 35385104

SN - 1462-0332

VL - 62

SP - 243

EP - 255

JO - Rheumatology (Oxford, England)

JF - Rheumatology (Oxford, England)

IS - 1

ER -

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

Abstract

Subject classification (UKÄ)

UN SDGs

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