Characterization and outcomes of 414 patients with primary SS who developed hematological malignancies.
Gabriela Hernández-MolinaBelchin KostovPilar Brito-ZerónArjan VissinkThomas MandlAnneline C HinrichsLuca QuartuccioChiara BaldiniRaphaele SerorAntonia SzántóDavid IsenbergRoberto GerliGunnel NordmarkAstrid RasmussenRoser Solans-LaqueBenedikt HofauerDamien SèneSandra G PasotoMaureen RischmuellerSonja PraprotnikTamer A GheitaDebashish DandaBerkan ArmağanYasunori SuzukiValeria ValimValerie Devauchelle-PensecSoledad RetamozoMarika KvarnstromAgata SebastianFabiola AtzeniRoberto GiacomelliSteven E CarsonsSeung-Ki KwokHideki NakamuraVirginia Fernandes Moça TrevisaniAlejandra Flores-ChávezXavier MarietteManuel Ramos-Casalsnull nullPublished in: Rheumatology (Oxford, England) (2022)
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%.