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

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...

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Bibliographic Details
Published in:International journal of molecular sciences 2022-11, Vol.23 (21), p.13392
Main Authors: Gaudio, Francesco, Masciopinto, Pierluigi, Bellitti, Emilio, Musto, Pellegrino, Arcuti, Elena, Battisti, Olga, Cazzato, Gerardo, Solombrino, Alessandra, Laddaga, Filomena Emanuela, Specchia, Giorgina, Maiorano, Eugenio, Ingravallo, Giuseppe
Format: Article
Language:English
Subjects:
Anemia
Antigens
Apoptosis
Asymptomatic
Bone marrow
Case Report
Cloning
Cytokines
Cytotoxicity
Diagnostic systems
Disease control
Gene expression
Genotype & phenotype
Health services
Humans
Immune system
Immunoproliferative diseases
Immunosuppressive agents
Infections
Janus Kinases
Kinases
Leukemia
Leukemia, Large Granular Lymphocytic - diagnosis
Leukemia, Large Granular Lymphocytic - drug therapy
Leukemia, Large Granular Lymphocytic - genetics
Lymphocytes
Lymphocytes T
Lymphoma
Medical prognosis
Mutation
Neutropenia
Pathogenesis
Peripheral blood
Phosphorylation
Senescence
Signal Transduction
STAT Transcription Factors
Stat3 protein
Thrombocytopenia
Tumors
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Summary: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.
ISSN:1422-0067
1661-6596
1422-0067
DOI:10.3390/ijms232113392