{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,2]],"date-time":"2025-11-02T09:17:26Z","timestamp":1762075046805,"version":"build-2065373602"},"reference-count":59,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2022,11,2]],"date-time":"2022-11-02T00:00:00Z","timestamp":1667347200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Large granular lymphocyte leukemia is a rare chronic lymphoproliferative disease of cytotoxic lymphocytes. The diagnosis, according to the WHO, is based on a persistent (&gt;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.<\/jats:p>","DOI":"10.3390\/ijms232113392","type":"journal-article","created":{"date-parts":[[2022,11,3]],"date-time":"2022-11-03T03:11:21Z","timestamp":1667445081000},"page":"13392","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Molecular Features and Diagnostic Challenges in Alpha\/Beta T-Cell Large Granular Lymphocyte Leukemia"],"prefix":"10.3390","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7817-1665","authenticated-orcid":false,"given":"Francesco","family":"Gaudio","sequence":"first","affiliation":[{"name":"Hematology Section, Department of Emergency and Transplantation, University of Bari Medical School, 70124 Bari, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pierluigi","family":"Masciopinto","sequence":"additional","affiliation":[{"name":"Hematology Section, Department of Emergency and Transplantation, University of Bari Medical School, 70124 Bari, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8959-5773","authenticated-orcid":false,"given":"Emilio","family":"Bellitti","sequence":"additional","affiliation":[{"name":"Section of Pathology, Department of Emergency and Organ Transplantation (DETO), University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3277-6594","authenticated-orcid":false,"given":"Pellegrino","family":"Musto","sequence":"additional","affiliation":[{"name":"Hematology Section, Department of Emergency and Transplantation, University of Bari Medical School, 70124 Bari, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elena","family":"Arcuti","sequence":"additional","affiliation":[{"name":"Hematology Section, Department of Emergency and Transplantation, University of Bari Medical School, 70124 Bari, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olga","family":"Battisti","sequence":"additional","affiliation":[{"name":"Hematology Section, Department of Emergency and Transplantation, University of Bari Medical School, 70124 Bari, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0325-4316","authenticated-orcid":false,"given":"Gerardo","family":"Cazzato","sequence":"additional","affiliation":[{"name":"Section of Pathology, Department of Emergency and Organ Transplantation (DETO), University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessandra","family":"Solombrino","sequence":"additional","affiliation":[{"name":"Section of Pathology, Department of Emergency and Organ Transplantation (DETO), University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Filomena Emanuela","family":"Laddaga","sequence":"additional","affiliation":[{"name":"Clinical Pathology Unit, AOUC Policlinico, Piazza G. Cesare, 11, 70124 Bari, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giorgina","family":"Specchia","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Bari \u201cAldo Moro\u201d, Piazza G. Cesare, 11, 70124 Bari, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0472-5338","authenticated-orcid":false,"given":"Eugenio","family":"Maiorano","sequence":"additional","affiliation":[{"name":"Section of Pathology, Department of Emergency and Organ Transplantation (DETO), University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4792-3545","authenticated-orcid":false,"given":"Giuseppe","family":"Ingravallo","sequence":"additional","affiliation":[{"name":"Section of Pathology, Department of Emergency and Organ Transplantation (DETO), University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,11,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1080\/10428194.2017.1339880","article-title":"Clinical features and treatment outcomes in large granular lymphocytic leukemia (LGLL)","volume":"59","author":"Sanikommu","year":"2017","journal-title":"Leuk. Lymphoma"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1720","DOI":"10.1038\/s41375-022-01620-2","article-title":"The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms","volume":"36","author":"Alaggio","year":"2022","journal-title":"Leukemia"},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Lamy, T., Moignet, A., and Loughran, T.P. (2017). Review Series T-CELL MALIGNANCIES LGL leukemia: From pathogenesis to treatment. 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