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

Gorodetskiy, Vadim; Vasilyev, Vladimir; Sidorova, Yu S; Бидерман, Б В; Купрышина, Н. А.; Vagida, Murad; Ryzhikova, N V; Sudarikov, Andrey

doi:10.3390/ijms232113345

Cited by 2 publications

(1 citation statement)

References 51 publications

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“…Sjogren's syndrome is an autoimmune disorder characterized by the infiltration of autoreactive T-and B cells into the salivary and lacrimal glands, resulting in inflammation and tissue damage. Few cases of Sjogren's syndrome occurring concurrently with T-LGL leukemia have been reported in the literature, and the causal relationship between Sjogren's syndrome and T-LGL leukemia is unclear [97]. One proposed mechanism involves the activation of IL-15.…”

Section: Sjogren's Syndromementioning

confidence: 99%

Large Granular Lymphocytic Leukemia: Clinical Features, Molecular Pathogenesis, Diagnosis and Treatment

Ullah,

Markouli,

Orland

et al. 2024

Cancers

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Large granular lymphocytic (LGL) leukemia is a lymphoproliferative disorder characterized by persistent clonal expansion of mature T- or natural killer cells in the blood via chronic antigenic stimulation. LGL leukemia is associated with specific immunophenotypic and molecular features, particularly STAT3 and STAT5 mutations and activation of the JAK-STAT3, Fas/Fas-L and NF-κB signaling pathways. Disease-related deaths are mainly due to recurrent infections linked to severe neutropenia. The current treatment is based on immunosuppressive therapies, which frequently produce unsatisfactory long-term responses, and for this reason, personalized approaches and targeted therapies are needed. Here, we discuss molecular pathogenesis, clinical presentation, associated autoimmune disorders, and the available treatment options, including emerging therapies.

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Section: Sjogren's Syndromementioning

confidence: 99%

Large Granular Lymphocytic Leukemia: Clinical Features, Molecular Pathogenesis, Diagnosis and Treatment

Ullah,

Markouli,

Orland

et al. 2024

Cancers

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The constitutive activation of STAT3 gene and its mutations are at the crossroad between LGL leukemia and autoimmune disorders

Semenzato,

Calabretto,

Teramo

et al. 2024

Blood Cancer J.

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Type T Large Granular Lymphocyte Leukemia (T-LGLL) is a chronic disorder characterized by the abnormal proliferation of clonal cytotoxic T cells. The intriguing association of T-LGLL with autoimmune and inflammatory diseases, the most prominent example being rheumatoid arthritis, raises questions about the underlying pathophysiologic relationships between these disorders which share several biological and clinical features, most notably neutropenia, which is considered as a clinical hallmark. Recent progress in molecular genetics has contributed to a better understanding of pathogenetic mechanisms, thus moving our knowledge in the field of LGL leukemias forward. Focusing on the constitutive activation of STAT3 pathway and the well-established role of STAT3 mutations in T-LGLL, we herein discuss whether the T cell clones occurring in comorbid conditions are the cause or the consequence of the immune-inflammatory associated events. Overall, this review sheds light on the intricate relationships between inflammation and cancer, emphasizing the importance of the STAT3 gene and its activation in the pathophysiology of these conditions. Gaining a deeper understanding of these underlying mechanisms seeks to pave the way for the development of novel targeted therapies for patients affected by inflammation-related cancers.

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

Cited by 2 publications

References 51 publications

Large Granular Lymphocytic Leukemia: Clinical Features, Molecular Pathogenesis, Diagnosis and Treatment

Large Granular Lymphocytic Leukemia: Clinical Features, Molecular Pathogenesis, Diagnosis and Treatment

The constitutive activation of STAT3 gene and its mutations are at the crossroad between LGL leukemia and autoimmune disorders

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