Large granular lymphocyte expansions in primary Sjögren’s syndrome: characteristics and outcomes

Baber, Alistair; Nocturne, Gaëtane; Krzysiek, Roman; Henry, J.; Belkhir, Rakiba; Mariette, Xavier; Séror, Raphaèle

doi:10.1136/rmdopen-2019-001044

Cited by 4 publications

(6 citation statements)

References 13 publications

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“…The fact that the LGL in this case, as well as in all others reported [ 7 - 9 ], was determined by CD8+ T cells could represent a further argument that CD8+ cells undergo important modifications in pSS, which should be taken in consideration. Indeed, in our case, the diagnosis of LGL was made together with that of pSS, suggesting that the two diseases share at least part of the pathogenesis, if not a common etiology, as has been suggested in a previous report [ 7 ].…”

Section: Discussionsupporting

confidence: 72%

“…LGL leukemia is a rare condition accounting for 2-3% of all mature lymphoid leukemias [ 6 ]. The finding of LGL in the context of autoimmune disorders is common, but it often occurs in rheumatoid arthritis or in the presence of less specific autoimmune features (such as positivity to autoantibodies, e.g., antinuclear antibodies); in pSS, less than 15 cases have been described [ 7 - 9 ].…”

Section: Discussionmentioning

confidence: 99%

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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

et al. 2020

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Section: Discussionsupporting

confidence: 72%

Section: Discussionmentioning

confidence: 99%

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

et al. 2020

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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%

“…Constitutive activation of the STAT3 pathway is a hallmark of T-LGL leukemia pathobiology [ 13 , 43 ], and STAT3 mutations were detected in 27–72% of patients with T-LGL leukemia [ 13 , 44 , 45 , 46 ]. To the best of our knowledge, STAT3 mutation studies have only been performed in three patients with SS-associated T-LGL leukemia [ 27 ]. In all of these cases, no STAT3 mutations were detected.…”

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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Type 2 hypersensitivity disorders, including systemic lupus erythematosus, Sjögren’s syndrome, Graves’ disease, myasthenia gravis, immune thrombocytopenia, autoimmune hemolytic anemia, dermatomyositis, and graft-versus-host disease, are THαβ-dominant autoimmune diseases

Huang,

Shih,

Hsieh

et al. 2024

Virulence

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The THαβ host immunological pathway contributes to the response to infectious particles (viruses and prions). Furthermore, there is increasing evidence for associations between autoimmune diseases, and particularly type 2 hypersensitivity disorders, and the THαβ immune response. For example, patients with systemic lupus erythematosus often produce anti-double stranded DNA antibodies and anti-nuclear antibodies and show elevated levels of type 1 interferons, type 3 interferons, interleukin-10, IgG1, and IgA1 throughout the disease course. These cytokines and antibody isotypes are associated with the THαβ host immunological pathway. Similarly, the type 2 hypersensitivity disorders myasthenia gravis, Graves’ disease, graft-versus-host disease, autoimmune hemolytic anemia, immune thrombocytopenia, dermatomyositis, and Sjögren’s syndrome have also been linked to the THαβ pathway. Considering the potential associations between these diseases and dysregulated THαβ immune responses, therapeutic strategies such as anti-interleukin-10 or anti-interferon α/β could be explored for effective management.

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Large granular lymphocyte expansions in primary Sjögren’s syndrome: characteristics and outcomes

Cited by 4 publications

References 13 publications

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

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

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

Type 2 hypersensitivity disorders, including systemic lupus erythematosus, Sjögren’s syndrome, Graves’ disease, myasthenia gravis, immune thrombocytopenia, autoimmune hemolytic anemia, dermatomyositis, and graft-versus-host disease, are THαβ-dominant autoimmune diseases

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