Article
Biochemistry & Molecular Biology
Francesco Gaudio, Pierluigi Masciopinto, Emilio Bellitti, Pellegrino Musto, Elena Arcuti, Olga Battisti, Gerardo Cazzato, Alessandra Solombrino, Filomena Emanuela Laddaga, Giorgina Specchia, Eugenio Maiorano, Giuseppe Ingravallo
Summary: Large granular lymphocyte leukemia is a rare chronic disease characterized by neutropenia, anemia, and thrombocytopenia. It is often associated with various autoimmune conditions and has an indolent course.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Immunology
Tao Zhao, Nan Hu, Xiaojuan Yu, Tao Su
Summary: Large granular T lymphocyte leukemia (T-LGLL) is a rare chronic lymphocyte leukemia characterized by clonal proliferation of T cells, which is related to STAT3 gene mutation and abnormal apoptosis pathway. Studies have suggested that exogenous and continuous stimulation, such as virus infection, may be related to the pathogenesis of T-LGLL.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Biochemistry & Molecular Biology
Vadim Gorodetskiy, Vladimir Vasilyev, Yulia Sidorova, Bella Biderman, Natalia Kupryshina, Murad Vagida, Natalya Ryzhikova, Andrey Sudarikov
Summary: This paper reports a large case series of 21 patients with primary and secondary SS associated with T-LGL leukemia, highlighting the importance of considering T-LGL leukemia in the diagnostic evaluation of SS patients, especially when neutropenia occurs. Elevated antinuclear antibody titers in patients with T-LGL leukemia may indicate the need for clinical assessment of SS.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Oncology
Kexin Ai, Minming Li, Ping Wu, Chengxin Deng, Xin Huang, Wei Ling, Ruohao Xu, Suxia Geng, Qihui Sun, Jianyu Weng, Xin Du
Summary: This study detailed the clinicopathological features of 10 cases of MDS concurrent with LGLL, showing the high frequency mutations in ASXL1 and STAG2 genes, as well as LGLL-associated mutations of STAT3 and STAT5b. Whole-exome sequencing and gene ontology analysis revealed changes in pathways upon progression from MDS to MDS-LGLL, with increased enrichment of H3K4 mono-methylation and decreased enrichment of ZAP-70 translocation to immunological synapse.
AMERICAN JOURNAL OF CANCER RESEARCH
(2021)
Article
Multidisciplinary Sciences
Shouguo Gao, Zhijie Wu, Bradley Arnold, Carrie Diamond, Sai Batchu, Valentina Giudice, Lemlem Alemu, Diego Quinones Raffo, Xingmin Feng, Sachiko Kajigaya, John Barrett, Sawa Ito, Neal S. Young
Summary: T-cell large granular lymphocyte leukemia (T-LGLL) is a lymphoproliferative disease characterized by reduced TCR diversity and deregulation of cell survival genes. After alemtuzumab treatment, apoptosis genes are upregulated, particularly in responders, and TCR diversity is further skewed. Analysis of single cell data provides insights into the clonal expansion and persistence mechanisms in T-LGLL.
NATURE COMMUNICATIONS
(2022)
Article
Hematology
Taekeun Park, Ja Min Byun, Dong-Yeop Shin, Youngil Koh, Junshik Hong, Sung-Soo Yoon, Yoon Hwan Chang, Inho Kim
Summary: Large granular lymphocytic (LGL) leukemia is a rare lymphoproliferative disorder derived from cytotoxic T lymphocytes or natural killer cells. This study investigated the clinical features and treatment outcomes of T-cell LGL leukemia (T-LGLL) patients. The results showed that cyclophosphamide, methotrexate, and cyclosporin A had similar response rates, while splenomegaly, thrombocytopenia, and high LGL number in peripheral blood smear were associated with treatment response. This study provides valuable information for clinical decision-making regarding T-LGLL treatment.
ANNALS OF HEMATOLOGY
(2023)
Review
Oncology
Qin Hu, Yunfei Li, Ying Zhang, Shusen Sun, Hui Wang, Zhiping Jiang, Sheng Deng
Summary: This case report describes a rare case of T-LGLL with a rare gamma delta type and fusion gene NPL-DHX9 rearrangement. The patient was successfully treated with cladribine, indicating that this regimen could be a promising therapeutic strategy for aggressive T-LGLL.
FRONTIERS IN ONCOLOGY
(2022)
Article
Oncology
Zachary Braunstein, Eric McLaughlin, Miguel Ruiz, Lai Wei, Naresh Bumma, Don Benson, Srinivas Devarakonda, Maria Chaudhry, Abdullah Khan, Francesca Cottini, Walter Hanel, Robert Baiocchi, Catherine Chung, Daniel Addison, Nina Couette, Alexa Meara, Wael Jarjour, Pierluigi Porcu, Anjali Mishra, John C. Reneau, Ashley E. Rosko, Jonathan E. Brammer
Summary: This study reported a group of patients with combined T-cell malignancies and plasma cell dyscrasias (PCD), including 26 patients. The results showed that there was no significant difference in survival for patients with CTCL or T-LGLL and concomitant PCD, but patients with PTCL and concomitant PCD had worse outcomes. Treatment directed at the T-cell malignancy resulted in the eradication of the PCD clone in some patients.
FRONTIERS IN ONCOLOGY
(2022)
Article
Medicine, General & Internal
Fawzi Abu Rous, Radhika Gutta, Rebecca Chacko, Philip Kuriakose, Vrushali Dabak
Summary: Large granular lymphocytic (LGL) leukemia is a rare chronic lymphoproliferative disorder that typically occurs in the sixth decade of life. Most cases of T-cell LGL leukemia (T-LGL) are associated with autoimmune disorders, and patients with T-LGL are generally asymptomatic but may present symptoms related to the immune system.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2022)
Review
Oncology
Katharine B. Moosic, Kusuma Ananth, Felipe Andrade, David J. Feith, Erika Darrah, Thomas P. Loughran
Summary: This review discusses the parallels and potential mechanisms between LGL leukemia and RA, suggesting that LGL may drive the development of RA. Further investigations are needed to better understand the relationship and develop targeted therapies for both disorders.
FRONTIERS IN ONCOLOGY
(2022)
Article
Oncology
Patricia Johansson, Teresa Laguna, Julio Ossowski, Vera Pancaldi, Martina Brauser, Ulrich Duehrsen, Lara Keuneke, Ana Queiros, Julia Richter, Jose Martin-Subero, Reiner Siebert, Brigitte Schlegelberger, Ralf Kueppers, Jan Duerig, Eva M. Murga Penas, Enrique Carillo-de Santa Pau, Anke K. Bergmann
Summary: This study identifies altered DNA methylation in T-LGLL cells compared to benign T cells, with BCL11B being highly differentially methylated. Although validation in a larger patient cohort is needed, BCL11B could be considered as a potential biomarker for this leukemia. Additionally, altered gene expression and hypermethylation of enhancer regions could serve as potential mechanisms for treatment of this disease.
CLINICAL EPIGENETICS
(2022)
Article
Hematology
Eun-Ji Choi, Young-Uk Cho, Eun-Hye Hur, Han-Seung Park, Yunsuk Choi, Jung-Hee Lee, Kyoo-Hyung Lee, Miyoung Kim, Sang-Hyun Hwang, Seongsoo Jang, Chan-Jeoung Park, Eul-Ju Seo, Je-Hwan Lee
Summary: This study compared the clinical and genetic characteristics of CCUS and lower-risk MDS, and found that patients with CCUS who had high-risk features had similar or worse overall survival compared to lower-risk MDS patients. These findings suggest that patients with certain clinical or genetic features of CCUS should be regarded and treated as lower-risk MDS.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Review
Oncology
Valentina Giudice, Matteo D'Addona, Nunzia Montuori, Carmine Selleri
Summary: Large granular lymphocyte (LGL) leukemia is a lymphoproliferative disease characterized by increased frequency of large-sized lymphocytes with typical T-cell receptor (TCR) expression, flow cytometry and molecular analysis are key for confirmation and monitoring of TCR clonality, V beta usage helps in identifying clonal TCR rearrangements, often associated with autoimmune disorders and other hematological conditions, potentially underlying silent LGL clones in other diseases.
Article
Oncology
Carlos Bravo-Perez, Salvador Carrillo-Tornel, Esmeralda Garcia-Torralba, Andres Jerez
Summary: Large granular lymphocyte leukemia (LGLL) is a chronic disease characterized by clonal expansions of mature cytotoxic T lymphocytes or NK cells. The diagnosis of LGLL is challenging due to the presence of reactive persistent clonal expansions that may fulfill the diagnostic criteria. LGLL has been associated with various conditions/disorders, and studying these associations can help understand the causality and determine the appropriate timing for targeted treatment.
Review
Oncology
Nina Couette, Wael Jarjour, Jonathan E. Brammer, Alexa Simon Meara
Summary: There exists a complex relationship between rheumatic diseases and cancer, involving chronic inflammation and malignant cell transformation. The association between Large Granular Lymphocyte leukemia and rheumatic diseases like rheumatoid arthritis has been well studied, but more research is needed to understand the molecular mechanisms underlying this connection.
FRONTIERS IN ONCOLOGY
(2022)
Letter
Hematology
Maria Siddiqui, Sergej Konoplev, Ghayas Issa, Hagop Kantarjian, Naval Daver, Farhad Ravandi, Tapan Kadia, Guilin Tang, Sa A. Wang, Beenu Thakral, L. Jeffrey Medeiros, Olga Pozdnyakova, Sherry Pierce, Guillermo Montalban-Bravo, Kelly Chien, Danielle Hammond, Koji Sasaki, Guillermo Garcia-Manero, Robert P. Hasserjian
AMERICAN JOURNAL OF HEMATOLOGY
(2023)
Article
Pathology
Ying-Ren Chen, Shan-Chi Yu, Ren-Ching Wang, Chih-Ling Lee, Hsiang-Lin Song, L. Jeffrey Medeiros, Chung-Tai Yue, Kung-Chao Chang
Summary: This study investigated 55 lymph node biopsy specimens and found that increased IgG4(+) plasma cells can be a nonspecific finding or a manifestation of IgG4-related disease. The study also found minimal differences between patients with lymphadenopathy who did and did not exhibit other manifestations of IgG4-related disease.
AMERICAN JOURNAL OF SURGICAL PATHOLOGY
(2023)
Article
Oncology
Yi Wang, Andres E. Quesada, Zhuang Zuo, L. Jeffrey Medeiros, C. Cameron Yin, Shaoying Li, Jie Xu, Gautam Borthakur, Yisheng Li, Chao Yang, Yasmin Abaza, Juehua Gao, Xinyan Lu, M. James You, Yizhuo Zhang, Pei Lin
Summary: Through a study of 107 patients with NPM1-mutated AML treated with risk-adapted therapy, it was found that patients with MDS-related gene mutations had a similar MMD rate compared to those without the mutations. However, the former group had a higher relapse rate and shorter progression-free survival.
Article
Oncology
Lianqun Qiu, Sa A. Wang, Guilin Tang, Wei Wang, Pei Lin, Jie Xu, C. Cameron Yin, Mahsa Khanlari, L. Jeffrey Medeiros, Shaoying Li
Summary: The six-point scoring system showed a sensitivity of 100% in distinguishing blastoid-HGBL and B-ALL. Using both scoring systems together improves the accuracy of classification of blastoid B-cell neoplasms to 99%.
Article
Biology
Zhuang Zuo, L. Jeffrey Medeiros, Sofia Garces, Mark J. Routbort, Chi Young Ok, Sanam Loghavi, Rashmi Kanagal-Shamanna, Fatima Zahra Jelloul, Guillermo Garcia-Manero, Kelly L. S. Chien, Keyur S. P. Patel, Rajyalakshmi Luthra, C. Cameron Yin
Summary: The genetic landscape of myeloid neoplasms has been elucidated by next-generation sequencing, revealing that multiple gene mutations are present in most cases. Understanding the patterns and interactions between these mutations can improve diagnosis and prognosis. SF3B1 and PHF6 mutations are known to have cooperative or mutually exclusive effects in the pathogenesis of myeloid neoplasms. This study reports the clinicopathologic and molecular features of 21 cases with double SF3B1 and PHF6 mutations, highlighting their rarity but presence in various myeloid neoplasms and their roles in early events and disease progression.
Article
Cell Biology
Mario L. Marques-Piubelli, Do Hwan Kim, L. Jeffrey Medeiros, Wei Lu, Khaja Khan, Lorena Isabel Gomez-Bolanos, Saxon Rodriguez, Edwin R. Parra, Chi Young Ok, Akanksha Aradhya, Luisa M. Solis, Yago L. Nieto, Raphael Steiner, Sairah Ahmed, Francisco Vega
Summary: CAR-T cell therapy results in decreased CD30 expression in refractory/relapsed classical Hodgkin lymphoma (CHL) patients, but certain levels of CD30 expression are still detectable.
Review
Pathology
Ling Zhang, Bijal Shah, Yumeng Zhang, Hammad Tashkandi, Wenbin Xiao, Sebastian Fernandez-Pol, Maria Vergara-Lluri, Mohammad Hussaini, Jinming Song, Jeffrey Lancet, Lynn Moscinski, Seongseok Yun, Chuanyi M. Lu, L. Jeffrey Medeiros, Guilin Tang
Summary: JAK2 rearrangement (JAK2-R) is a rare subtype of acute lymphoblastic leukemia (ALL) and mainly occurs in adult males, with a B-cell lineage. Patients with JAK2-R ALL often present with marked leukocytosis and hypercellular bone marrow. Survival rates for JAK2-R ALL are relatively low compared to other subtypes of ALL. The absence of eosinophilia and bone marrow fibrosis differentiate JAK2-R ALL from other myeloid/lymphoid neoplasms.
Article
Pathology
Ji Yuan, Hui Liu, Shimin Hu, Roberto N. Miranda, Xinjie Xu, Michael G. Bayerl, Cody J. Artymiuk, Holly Berg, Rebecca L. King, Min Shi, Rong He, David Viswanatha, L. Jeffrey Medeiros, Ellen D. Mcphail
Summary: This study reports 5 adult patients with FL and FL/DLBCL with concurrent BCL2 and IRF4 rearrangements. The clinicopathologic and mutational features of these patients are more akin to FL and DLBCL, and should not be characterized as large B-cell lymphoma with IRF4 rearrangement.
Meeting Abstract
Medicine, Research & Experimental
Fnu Aakash, L. Jeffrey Medeiros, Lianqun Qiu, Jie Xu, Pei Lin, Roberto Miranda, Beenu Thakral, M. James You, Shaoying Li
LABORATORY INVESTIGATION
(2023)
Meeting Abstract
Medicine, Research & Experimental
Okechukwu Nwogbo, Francis San Lucas, Hyvan Dang, Mario L. Marques-Piubelli, Amy Ayers, Hector Alvarez, Rajyalakshmi Luthra, Loretta Nastoupil, Christopher Flowers, L. Jeffrey Medeiros, Keyur Patel, Francisco Vega
LABORATORY INVESTIGATION
(2023)
Meeting Abstract
Medicine, Research & Experimental
Daniel Rivera, Shuyu E, Wei Cui, Qian-Yun Zhang, Rajan Dewar, Huan-You Wang, Zhihong Hu, Wei Wang, L. Jeffrey Medeiros, Shimin Hu
LABORATORY INVESTIGATION
(2023)
Meeting Abstract
Medicine, Research & Experimental
E. Shuyu, Meng Liu, Hong Fang, Wei Wang, Guilin Tang, L. Jeffrey Medeiros, Shimin Hu
LABORATORY INVESTIGATION
(2023)
Meeting Abstract
Medicine, Research & Experimental
E. Shuyu, L. Jeffrey Medeiros, Shaoying Li, Sa Wang, Pei Lin, Wei Wang, Swami Iyer, Luis Malpica-Castillo, C. Cameron Yin, Guilin Tang, Zhenya Tang, M. James You, Jie Xu
LABORATORY INVESTIGATION
(2023)
Meeting Abstract
Medicine, Research & Experimental
Zhenya Tang, Guilin Tang, Gokce Toruner, Su Yang, Wei Wang, Hong Fang, Hanadi El Achi, Beenu Thakral, Jie Xu, C. Cameron Yin, M. James You, Roberto Miranda, Joseph Khoury, L. Jeffrey Medeiros
LABORATORY INVESTIGATION
(2023)
Article
Biology
Hillary P. Esplen, Richard K. Yang, Awdhesh Kalia, Zhenya Tang, Guilin Tang, L. Jeffrey Medeiros, Gokce A. Toruner
Summary: There is a strong correlation between recurrent somatic copy number alterations (SCNAs) and gene expression levels in high-grade ovarian serous carcinoma (HGOSC). Several genes with highly correlated SCNA and mRNA expression levels are associated with clinicopathological parameters such as age, disease stage, and survival.