Brito-Zerón P et al. - Neutropenia should be considered a relevant hematologic finding of primary Sjögren's syndrome (SS), due both to its elevated prevalence and to its clinical significance. Methods
Prevalence of neutropenia was analyzed in pts (n=300) with primary SS to study its association with clinical and immunological disease expression and adverse outcomes
Results
33% of pts had neutropenia during the f/u, which was related to neoplasia or drugs in 3% pts and was considered idiopathic in the remaining 30%
Pts with neutropenia had a lower mean age at diagnosis of SS and a higher prevalence of anti-Ro/La Ab, RF and low C4 levels vs w/o neutropenia
Pts with neutropenia had a higher incidence of hospital admission caused by infection
Agranulocytosis was found in 2% of pts, predominantly related to neoplasia
1% of pts with SS-related neutropenia developed large granular lymphocyte T-cell leukemia