Article
Oncology
Giulia Pozzi, Cecilia Carubbi, Giuliana Gobbi, Sara Tagliaferri, Prisco Mirandola, Marco Vitale, Elena Masselli
Summary: The detection of CCR2(+)CD34(+) cells by flow cytometry has good diagnostic accuracy in distinguishing between different MPN subtypes with varying degrees of bone marrow fibrosis, particularly for differentiating between ET and prePMF as well as between prePMF and overtPMF.
FRONTIERS IN ONCOLOGY
(2022)
Editorial Material
Hematology
Heike L. Pahl
Summary: The identification of sentinel mutations in MPN patients, which significantly increase the risk of leukemic transformation, even when not present in leukemic cells, is a crucial advancement in understanding and managing the disease. These mutations act as early indicators of potential disease progression, similar to canaries in a coal mine.
Editorial Material
Hematology
Ana Rio-Machin, Jude Fitzgibbon
Summary: In this study, Li et al and Duployez et al investigate the clinical and genetic landscape of DDX41 germline variants in patients with acute myeloid leukemia (AML). They find that AML with DDX41 germline variants represents a distinct subtype characterized by late-onset disease, normal karyotype, male sex skewing, and favorable outcome.
Article
Developmental Biology
Wei Zhang, Jiaojiao Yu, Guoquan Fu, Jianying Li, Huarong Huang, Jing Liu, Dongliang Yu, Mengsheng Qiu, Feixue Li
Summary: This study provides the first genetic evidence that the ISL1/SHH/CXCL12 axis regulates myoblast migration during tongue development. Deletion of Isl1 in the mouse mandibular epithelium leads to defects in myoblast migration, which is regulated by the Shh/Wnt5a cascade. Cxcl12+ mesenchymal cells in Isl1ShhCre embryos fail to migrate to the distal region and instead accumulate in the proximal domain of the mandible. This is due to the concentration-dependent bidirectional cue of CXCL12, which attracts myoblast invasion at low concentrations but repels it at high concentrations.
Article
Medicine, Research & Experimental
Can Veysel Soroglu, Ildeniz Uslu-Bicak, Selin Fulya Toprak, Akif Selim Yavuz, Selcuk Solzer
Summary: This study evaluated the effect of different oxygen concentrations on HIF-1a and NOS3 gene expression to determine the effect of the bone marrow microenvironment on JAK2V617F positive Philadelphia chromosome negative (Ph-) MPNs.
ADVANCES IN MEDICAL SCIENCES
(2023)
Article
Chemistry, Medicinal
Rhiannon Morris, Liesl Butler, Andrew Perkins, Nadia J. Kershaw, Jeffrey J. Babon
Summary: LNK is an adaptor protein that regulates cytokine signaling and constrains the activation of the JAK-STAT pathway. Mutations in LNK can lead to increased signaling and contribute to various hematological and inflammatory diseases.
Review
Biochemistry & Molecular Biology
Francisca Ferrer-Marin, Ernesto Jose Cuenca-Zamora, Pedro Jesus Guijarro-Carrillo, Raul Teruel-Montoya
Summary: In patients with chronic Philadelphia chromosome-negative myeloproliferative neoplasms (MPN), leukocytes play a significant role in thrombosis. Neutrophils and monocytes in MPN patients show a pro-coagulant activated phenotype and release extracellular neutrophil traps (NETs) that contribute to platelet activation and coagulation mechanisms. Therapeutic strategies targeting NETs may reduce thrombotic complications in these patients.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Immunology
Sofia R. Gardeta, Eva M. Garcia-Cuesta, Gianluca D'Agostino, Blanca Soler Palacios, Adriana Quijada-Freire, Pilar Lucas, Jorge Bernardino de la Serna, Carolina Gonzalez-Riano, Coral Barbas, Jose Miguel Rodriguez-Frade, Mario Mellado
Summary: The local lipid environment plays an important role in regulating CXCR4 organization and function, and modulating chemokine-triggered directed cell migration. Treatment of T cells with bacterial sphingomyelinase alters the lipid composition of the cell membrane, affecting membrane fluidity and CXCR4 nanoclustering and dynamics.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Biochemistry & Molecular Biology
Dominik Kiem, Sandro Wagner, Teresa Magnes, Alexander Egle, Richard Greil, Thomas Melchardt
Summary: Philadelphia chromosome negative myeloproliferative neoplasms consist of polycythemia vera, essential thrombocytosis, and primary myelofibrosis. Mutations in JAK2, MPL, or CALR characterize these disorders, with additional mutations leading to myeloid cell lineages expansion and marrow fibrosis in PMF. Chronic inflammation plays a significant role in the disease initiation and progression, with neutrophilic granulocytes playing a major role in the pathogenesis of thromboembolic events.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Biochemistry & Molecular Biology
Dragana Markovic, Irina Maslovaric, Dragoslava Djikic, Vladan P. Cokic
Summary: This article explores the important role of neutrophils in MPN and the potential link between apoptosis defects and chronic inflammation.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Letter
Oncology
Antonella Zagaria, Francesco Tarantini, Paola Orsini, Luisa Anelli, Cosimo Cumbo, Nicoletta Coccaro, Giuseppina Tota, Crescenzio Francesco Minervini, Elisa Parciante, Maria Rosa Conserva, Immacolata Redavid, Alessandra Ricco, Immacolata Attolico, Giorgina Specchia, Pellegrino Musto, Francesco Albano
Summary: This study investigated the genomic and clinical features of 80 erythrocytosis patients and found that male patients with idiopathic erythrocytosis and normal EPO levels may be the best candidates for searching for the JAK2 GGCC_46/1 haplotype and CALR rs1049481_G allele.
EXPERIMENTAL HEMATOLOGY & ONCOLOGY
(2022)
Article
Cell Biology
Jaturawat Pawinwongchai, Panchalee Jangprasert, Nungruthai Nilsri, Nipan Israsena, Ponlapat Rojnuckarin
Summary: JAK2V617F expression in iPSCs preferentially promoted megakaryocytes with a signaling profile distinctive from JAK2exon12 expression. Treatments with interferon alpha or arsenic trioxide preferentially suppressed the mutated over wild-type JAK2 signaling. This iPSC model is helpful in mechanistic studies and novel therapy screen for myeloproliferative neoplasm.
Review
Oncology
Marco Pizzi, Giorgio Alberto Croci, Marco Ruggeri, Silvia Tabano, Angelo Paolo Dei Tos, Elena Sabattini, Umberto Gianelli
Summary: Myeloproliferative neoplasms (MPNs) are a group of clonal hematopoietic stem cell disorders, characterized by increased proliferation of the myeloid lineages in the bone marrow. The classification and diagnostic criteria of MPNs have evolved over time to include a subset of cases that cannot be clearly classified, such as early phase MPNs, terminal fibrotic MPNs, and those associated with other disorders. Further research is needed to fully understand and classify these unclassifiable MPNs.
Article
Oncology
Sarah Jungius, Franziska C. Adam, Kerstin Grosheintz, Michael Medinger, Andreas Buser, Jakob R. Passweg, Joerg P. Halter, Sara C. Meyer
Summary: Myelofibrosis (MF) is a rare hematopoietic stem cell disorder. Allogeneic hematopoietic cell transplantation (HCT) is a potential curative therapy for advanced MF, but MF patients have delayed engraftment compared to AML patients, leading to complications. This study investigated engraftment dynamics in MF patients and found that delayed neutrophil and platelet engraftment in MF is related to both host and treatment-related factors.
FRONTIERS IN ONCOLOGY
(2023)
Review
Health Care Sciences & Services
Heather R. Wolfe, Mitchell E. Horwitz, Lindsay A. M. Rein
Summary: Primary myelofibrosis is a BCR-ABL1 negative myeloproliferative neoplasm characterized by clonal proliferation of myeloid cells. Allogenic hematopoietic stem cell transplantation may provide a safe and effective curative option for a broader range of primary myelofibrosis patients in the future.
JOURNAL OF PERSONALIZED MEDICINE
(2022)