Introduction: T-LGL is a rare chronic lymphoproliferative disorder. It is often associated with a spectrum of clinical manifestations, resulting in significant clinical course and outcome heterogeneity. Objectives: This study aims to evaluate the clinicopathologic factors impacting T-LGL survival. Methods: We compiled a pooled database of T-LGL cases, constructed Kaplan-Meier survival curves, and used the Cox proportional hazards model and Log-rank tests to assess the influence of demographic and clinicopathologic factors on overall survival (OS). Results: A total of 132 patients were included in the analysis. The median age was 57, with no sexual preponderance. Autoimmune disorders were present in 56%, and TCR was clonal in 95% (TCRαβ+ 70%). While 60% were alive at 15 years, the median DFS was 36 months. Constitutional symptoms, infections, splenomegaly, and response-less than CR were detrimental to OS. OS was not impacted by age, blood counts including LGL, %BM involvement, ↑LDH, RF/ANA/DAT+, presence of monoclonal gammopathy or hepatomegaly, and type of TCR. OS was numerically worse in Males, LN+, CD4-, CD56+, and ↓albumin, but better in the presence of Rheumatoid arthritis, though none researched statistical significance. While there was no additive impact of combined chemotherapy on OS, including CSA and MTX in the course of the disease improved OS. Conclusions: This study identifies key clinical factors and treatment modalities that are major determinants of OS in this rare disease. These findings provide valuable insights into the prognostic determinants and optimal management strategies for T-LGL.
