Large granular lymphocytes (LGL) in primary Sjögren syndrome (pSS): immunophenotype and review on the pathological role of T cells in pSS

Tavarozzi, Rita; Carulli, Giovanni; Manzato, Enrica; Sammuri, Paola; Ciabatti, Elena; Petrini, Mario

doi:10.5045/br.2020.2020052

Cited by 2 publications

(3 citation statements)

References 22 publications

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“…Surprisingly, Friedman et al, in a cohort of 13 patients with SS-associated T-LGL leukemia, observed clonal dominant expansion of CD4+ T-lymphocytes in 4 (31%) patients [ 24 ]. Moreover, out of five patients with pSS-associated T-LGL leukemia, four patients had the typical CD8+ phenotype [ 25 , 27 , 28 ], and in one patient T-LGLs coexpressed CD4 and CD8 [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

“…Apparently, the first description of SS with LGL leukemia was presented by Goske et al (1980), who reported on a 52-year-old woman with SS, absolute lymphocytosis, and neutropenia [18]. We found only 39 cases of T-LGL leukemia in SS patients in PubMed to date [7,[19][20][21][22][23][24][25][26][27][28]. Of these, 31 cases of T-LGL leukemia were associated with pSS, 2 cases with sSS (one patient had RA and another had limited scleroderma), and in 6 cases, no information was provided.…”

Section: Discussionmentioning

confidence: 99%

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Clinical Study of the Relationship between Sjögren Syndrome and T-Cell Large Granular Lymphocytic Leukemia: Single-Center Experience

Gorodetskiy

Vasilyev²,

Sidorova

et al. 2022

IJMS

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The relationship between Sjögren syndrome (SS) and T-cell large granular lymphocytic (T-LGL) leukemia remains unclear. In this paper, we report for the first time a large case series of 21 patients with primary and secondary SS associated with T-LGL leukemia. Our results suggest the importance of considering T-LGL leukemia in the diagnostic evaluation of SS patients, particularly when neutropenia occurs. We also postulate that elevated antinuclear antibody titers in patients with T-LGL leukemia indicate the need for the clinical assessment of SS. To assess whether SS affects the frequency of the signal transducer and activator of transcription 3 (STAT3) gene mutations in T-LGL leukemia, we examined STAT3 mutations by next-generation sequencing in two cohorts of patients: with SS-associated T-LGL leukemia and T-LGL leukemia in the setting of rheumatic diseases but without SS. While our results suggest that SS, per se, is not associated with an increased frequency of STAT3 mutations in T-LGL leukemia, further studies are needed to better assess the role of the STAT pathway in the development of concomitant SS and T-LGL leukemia.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Clinical Study of the Relationship between Sjögren Syndrome and T-Cell Large Granular Lymphocytic Leukemia: Single-Center Experience

Gorodetskiy

Vasilyev²,

Sidorova

et al. 2022

IJMS

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“…In a cohort of patients with SS, stimulation of peripheral blood monocytes by IL-6 revealed increased activation of STAT3 ( 65 ). A phenotype of LGL that has been described in association with SS represents the T emRA subset, which can be seen in the setting of chronic inflammation, but is classically associated with low cell proliferation and high cell death rate compared to LGLs which have prolonged survival due to STAT pathway activation ( 66 ). Overall, these findings highlight overlap between chronic inflammation and autoimmunity as well as the difficulty associated with determining which process is the primary etiology.…”

Section: Activation Of the Jak-stat Pathwaymentioning

confidence: 99%

Pathogenesis and Treatment of T-Large Granular Lymphocytic Leukemia (T-LGLL) in the Setting of Rheumatic Disease

2022

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A complex relationship exists between rheumatic diseases and cancer. This delicate balance between chronic inflammation and malignant cell transformation in hematologic neoplasms has been observed, but is not well defined. Large Granular Lymphocyte (LGL) leukemia is at the intersection of a clonal lymphoproliferative disease, chronic inflammation, and autoimmunity. The association between rheumatoid arthritis (RA) and the spectrum of Felty’s Syndrome is well-known. Other rheumatic disorders have been reported including systemic lupus erythematosus (SLE), Sjogren’s Syndrome (SS), vasculitis, Behcet’s Disease (BD) and systemic sclerosis. The association between T-LGLL and rheumatic disease pathogenesis has been hypothesized, but has not yet been fully understood. Components of a shared pathogenesis includes chronic antigen stimulation, JAK-STAT pathway activation and overlap of various cytokines. We will summarize current knowledge on the molecular understanding between T-LGLL and rheumatic disease. There are many potential areas of research to help meet this need and lead to development of targeted therapeutic options.

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Large granular lymphocytes (LGL) in primary Sjögren syndrome (pSS): immunophenotype and review on the pathological role of T cells in pSS

Abstract: We would like to thank Dr. Linda Carli for her help in ensuring a correct follow-up of the patient.

Cited by 2 publications

References 22 publications

Clinical Study of the Relationship between Sjögren Syndrome and T-Cell Large Granular Lymphocytic Leukemia: Single-Center Experience

Clinical Study of the Relationship between Sjögren Syndrome and T-Cell Large Granular Lymphocytic Leukemia: Single-Center Experience

Pathogenesis and Treatment of T-Large Granular Lymphocytic Leukemia (T-LGLL) in the Setting of Rheumatic Disease

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