Skip to main content

Lund University Publications

LUND UNIVERSITY LIBRARIES

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

Hernández-Molina, Gabriela ; Kostov, Belchin ; Brito-Zerón, Pilar ; Vissink, Arjan ; Mandl, Thomas LU orcid ; Hinrichs, Anneline C ; Quartuccio, Luca ; Baldini, Chiara ; Seror, Raphaele and Szántó, Antonia , et al. (2022) In Rheumatology (Oxford, England) 62(1). p.243-255
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 =... (More)

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%.

(Less)
Please use this url to cite or link to this publication:
author
; ; ; ; ; ; ; ; and , et al. (More)
; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; and (Less)
author collaboration
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Rheumatology (Oxford, England)
volume
62
issue
1
pages
243 - 255
publisher
Oxford University Press
external identifiers
  • scopus:85139097101
  • pmid:35385104
ISSN
1462-0332
DOI
10.1093/rheumatology/keac205
language
English
LU publication?
yes
additional info
© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.
id
f9c4b5df-b8b7-4b1e-b939-78b82885df85
date added to LUP
2022-04-10 16:18:31
date last changed
2025-03-09 02:28:05
@article{f9c4b5df-b8b7-4b1e-b939-78b82885df85,
  abstract     = {{<p>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.</p><p>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.</p><p>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).</p><p>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%.</p>}},
  author       = {{Hernández-Molina, Gabriela and Kostov, Belchin and Brito-Zerón, Pilar and Vissink, Arjan and Mandl, Thomas and Hinrichs, Anneline C and Quartuccio, Luca and Baldini, Chiara and Seror, Raphaele and Szántó, Antonia and Isenberg, David and Gerli, Roberto and Nordmark, Gunnel and Rasmussen, Astrid and Solans-Laque, Roser and Hofauer, Benedikt and Sène, Damien and Pasoto, Sandra G and Rischmueller, Maureen and Praprotnik, Sonja and Gheita, Tamer A and Danda, Debashish and Armağan, Berkan and Suzuki, Yasunori and Valim, Valeria and Devauchelle-Pensec, Valerie and Retamozo, Soledad and Kvarnstrom, Marika and Sebastian, Agata and Atzeni, Fabiola and Giacomelli, Roberto and Carsons, Steven E and Kwok, Seung-Ki and Nakamura, Hideki and Fernandes Moça Trevisani, Virginia and Flores-Chávez, Alejandra and Mariette, Xavier and Ramos-Casals, Manuel}},
  issn         = {{1462-0332}},
  language     = {{eng}},
  number       = {{1}},
  pages        = {{243--255}},
  publisher    = {{Oxford University Press}},
  series       = {{Rheumatology (Oxford, England)}},
  title        = {{Characterization and outcomes of 414 patients with primary SS who developed hematological malignancies}},
  url          = {{http://dx.doi.org/10.1093/rheumatology/keac205}},
  doi          = {{10.1093/rheumatology/keac205}},
  volume       = {{62}},
  year         = {{2022}},
}