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Hematologic manifestations and predictors of lymphoma development in primary Sjogren syndrome: clinical and pathophysiologic aspects
Medicine, 09/14/09

Baimpa E et al. – Anemia of chronic disease and hypergammaglobulinemia are common hematologic manifestations at diagnosis and during the course of pSS. Neutropenia and cryoglobulinemia at diagnosis are significantly associated with an increased risk of lymphoma development.

Methods

Retrospective study of 536 consecutive patients
Prevalence of hematologic abnormalities and their associations with various disease manifestations in pSS assessed
Risk factors for the development of non-Hodgkin lymphoma (NHL) overall and by subtype identified

Results

Anemia of chronic disease and hypergammaglobulinemia were the most prevalent hematologic manifestations during the course of pSS.
Lymphoma was diagnosed in 7.5% of patients
Marginal zone B-cell lymphomas (MZBCLs) were the predominant histologic type 65%
while diffuse large B-cell lymphomas (DLBCLs) accounted for 17.5% of all cases
Development of NHL in patients with pSS could be predicted by the presence of simple clinical and laboratory factors at diagnosis: neutropenia , cryoglobulinemia , splenomegaly , lymphadenopathy , and low C4 levels
Patients carrying these factors had more than 5-fold increased risk of NHL compared to patients with no risk factors
Disease characteristics could predict subsequent development of MZBCL
Presence of lymphocytopenia at diagnosis served as risk factor for the development of a non-MZBCL