Hematologic manifestations and predictors of XXXX development in primary Sj  gren syndrome: clinical and pathophysiologic aspects.

@entity1 @entity3329 @entity417 @entity882 @entity706 @entity2905 @entity1283 @entity2133 @entity14033 @entity102 @entity4125 @entity1503 @entity1398 @entity1892 @entity4754 @entity381 @entity1893
The diverse @entity1503 of primary Sj  gren syndrome (pSS) have not been systematically investigated, and their prognostic relevance remains unclear. We conducted a retrospective study of 536 consecutive @entity1 followed in our institution to assess the prevalence of @entity1503 and their associations with various disease manifestations in pSS. We also aimed to identify risk factors for the development of @entity1893 ( @entity1893 ) overall and by subtype. @entity417 of @entity102 and @entity4754 were the most prevalent @entity1503 encountered at diagnosis and during the course of pSS. Univariate analysis between @entity1503 and glandular manifestations revealed a statistically significant correlation between @entity2905 and @entity1283 (p = 0.002), as well as between @entity1398 and @entity2133 (p = 0.019). @entity417 , @entity2905 , @entity882 , @entity4754 , the presence of monoclonal serum proteins, and @entity14033 correlated significantly with the presence of extraglandular symptoms such as palpable @entity4125 , @entity3329 , and @entity381 . @entity706 was diagnosed in 7.5% (95% confidence interval [CI], 5.4%-10%) of @entity1 . Marginal zone @entity1892 (MZBCLs) were the predominant histologic type (65%; 95% CI, 48.3%-79.4%), while diffuse large @entity1892 (DLBCLs) accounted for 17.5% (95% CI, 7.3%-32.8%) of all cases. The development of @entity1893 in @entity1 with pSS could be predicted by the presence of simple clinical and laboratory factors at diagnosis: @entity1398 (p = 0.041), @entity14033 (p = 0.008), @entity381 (p = 0.006), @entity3329 (p = 0.021), and low C4 levels (p = 0.009). @entity1 carrying any of these factors had a more than 5-fold increased risk of @entity1893 compared to @entity1 with no risk factors at all. The above set of disease characteristics could predict subsequent development of MZBCL; the presence of @entity2905 (p = 0.044) at diagnosis served as a risk factor for the development of a non-MZBCL, most commonly DLBCL. @entity417 of @entity102 and @entity4754 are common @entity1503 at diagnosis and during the course of pSS. @entity1398 and @entity14033 at diagnosis are significantly associated with an increased risk of @entity706 development.
