Abstract
7542 Background: Large granular lymphocyte (LGL) disorders represent a spectrum of aberrant T-cell or natural killer cell lymphocytic proliferations. LGLL is classically associated with autoimmune conditions and bone marrow (BM) failure disorders. SM has been reported in association with LGLL in about 10%. The aim of this study is to evaluate the impact of SM on the clinical course of LGLL. Methods: This is a retrospective study of LGLL patients (pts) evaluated at Moffitt Cancer Center between January 1995 and May 2016. The diagnostic clinico-pathological criteria consisted of LGL count > 0.5 k/μL with T-cell receptor gene rearrangement. Lower absolute number of clonal circulating LGLs with characteristic immunophenotype associated with BM involvement, cytopenias, splenomegaly and/or associated symptoms were also diagnostic. Pts with myelodysplastic syndrome were excluded. Survival analysis was performed using the Kaplan-Meier method with log-rank test. Chi-square and T-test were used to analyze association among various variables. Significant P-value was considered < 0.05. Results: Out of 668 screened pts with LGL expansions in peripheral blood, 261 met criteria for LGLL. SM were present in 44% (116/261) of LGLL pts, of which 38% were hematological and 80% arose prior to onset of LGLL. Most common solid SM included skin cancer (14%), prostate cancer (12%), and breast cancer (12%), while most common hematological SM consisted of non-Hodgkin lymphoma (17%) and chronic leukemia (14%). 5-year overall survival (OS) for all LGLL pts was 75% and 10-year OS 63%. There was a statistically significant difference in 5-year OS between LGLL pts with SM compared to without (p = 0.049), but no difference between both groups in median OS or 10-year OS. Pts diagnosed with SM prior to LGLL had worse 5-year OS (p = 0.031) and 10-year OS (p = 0.05) compared to all other LGLL pts. Conclusions: This study showed that the frequency of SM is higher than previously described, especially with onset prior to diagnosis of LGLL. Even though median age of LGLL is around 60 years, it appears that age itself cannot explain this phenomenon. Our results suggest that having a SM is a poor prognostic factor in LGLL patients.
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