The Value of Flow Cytometry Clonality in Large Granular Lymphocyte Leukemia

Giudice, Valentina; D’Addona, Matteo; Montuori, Nunzia; Selleri, Carmine

doi:10.3390/cancers13184513

Cited by 5 publications

(3 citation statements)

References 113 publications

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“…The post-thymectomy persistence of oligoclonal T cells with Vβ1 in thymoma-associated PRCA, described in a previous study [19], may also support the pathophysiological relevance of Vβ1 in PRCA. A systematic review of 533 patients with T-LGLL, including 80% of CD8+LGLL and 8.4% of PRCA, shows that the most frequent expanded Vβ family was Vβ13.1 (12.5%), while 5.7% had a dominant Vβ1 expansion [20]. Although a simple comparison across different cohorts is difficult, however, the predominance of Vβ1 in our extended cohort may be valid as an immunophenotypic characteristics of PRCA.…”

Section: Discussionmentioning

confidence: 77%

T cell clonal expansion and STAT3 mutations: a characteristic feature of acquired chronic T cell-mediated pure red cell aplasia

Kawakami

Yamane

et al. 2022

Int J Hematol

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Acquired chronic pure red cell aplasia (PRCA) develops idiopathically or in association with other medical conditions, including T cell large granular lymphocytic leukemia (T-LGLL) and thymoma. T cell dysregulation is considered a cardinal pathogenesis of PRCA, but genetic-phenotypic associations in T cell abnormalities are largely unclear.We evaluated an extended cohort of 90 patients with acquired PRCA, including 26 with idiopathic, 36 with T-LGLL-associated and 15 with thymoma-associated PRCA, for their T cell immunophenotypes, clonalities and STAT3 mutations. TCR repertoire skewing of CD8 + T cells was detected in 37.5% of idiopathic, 66.7% of T-LGLLassociated and 25% of thymoma-associated PRCA patients, and restriction to Vβ1 was most prominent (41%). Clonalities of TCRβ or γ chain and STAT3 mutational status were statistically associated (P=0.0398), and they were detected in all three subtypes.The overall response rate to cyclosporin A was 73.9%, without significant difference by subtypes nor STAT3 mutational status. The T cell dysregulations, such as TCR repertoire skewing with predominant Vβ1 usage, clonality and STAT3 mutations, were frequently found across the subtypes, and the close associations between them suggest that these T cell derangements reflect a common pathophysiological mechanism among these PRCA subtypes.

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Section: Discussionmentioning

confidence: 77%

T cell clonal expansion and STAT3 mutations: a characteristic feature of acquired chronic T cell-mediated pure red cell aplasia

Kawakami

Yamane

et al. 2022

Int J Hematol

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“…T-LGL leukemias have a constitutive mature post-thymic phenotype. The most frequent phenotype is of constitutively activated T cells (CD3+, TCR αβ+, CD4−, CD5dim, CD8+, CD16+, CD27−, CD28−, CD45R0−, CD45RA+, and CD57+), which are aggressive and generally associated with Stat5b mutations [ 3 , 16 ].…”

Section: Cytologymentioning

confidence: 99%

Molecular Features and Diagnostic Challenges in Alpha/Beta T-Cell Large Granular Lymphocyte Leukemia

Gaudio

Masciopinto

Bellitti

et al. 2022

IJMS

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Large granular lymphocyte leukemia is a rare chronic lymphoproliferative disease of cytotoxic lymphocytes. The diagnosis, according to the WHO, is based on a persistent (>6 months) increase in the number of LGL cells in the peripheral blood without an identifiable cause. A further distinction is made between T-LGL and NK-LGL leukemia. The molecular sign of LGL leukemia is the mutation of STAT3 and other genes associated with the JAK/STAT pathway. The most common clinical features are neutropenia, anemia, and thrombocytopenia, and it is often associated with various autoimmune conditions. It usually has an indolent course. Due to the rarity of the disease, no specific treatment has yet been identified. Immunosuppressive therapy is used and may allow for disease control and long-term survival, but not eradication of the leukemic clone. Here, we discuss the clinical presentation, diagnostic challenges, pathophysiology, and different treatment options available for alpha/beta T-LGL leukemia, which is the most common disease (85%), in order to better understand and manage this often misunderstood disease.

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“…Clonality can also be assessed by flow cytometry for different TCR chain domains (Vβ, Vγ, Vδ) using various antibodies. The current Vβ mAbs panel covers 65% of the Vβ spectrum ( 15 ). Detection of γδTCR and its subtypes (Vδ1 and Vδ2) at protein level by flow cytometry represents a fast practical method for determining the clonality of γδ T-cells ( 16 ).…”

Section: Introductionmentioning

confidence: 99%