The Effect of Fedratinib, a Selective Inhibitor of Janus Kinase 2, on Weight and Metabolic Parameters in Patients with Intermediate- or High-risk Myelofibrosis
Myelofibrosis (MF) is a chronic myeloproliferative neoplasm (MPN) characterized by hematopoietic stem cell-derived clonal myeloproliferation, abnormal cytokine expression, ineffective hematopoiesis, bone marrow fibrosis, splenomegaly, debilitating constitutional symptoms, increased risk of leukemic transformation, and shortened survival.1,2 Currently, two Janus-associated kinase (JAK) inhibitors, ruxolitinib and fedratinib, are approved for the treatment of MF based on their ability to reduce splenomegaly and MF symptom burden. (Source: Clinical Lymphoma, Myeloma and Leukemia)
Source: Clinical Lymphoma, Myeloma and Leukemia - January 7, 2022 Category: Hematology Authors: Douglas Tremblay, Lara Cavalli, Oumar Sy, Shelonitda Rose, John Mascarenhas Source Type: research

Retinal drusen in patients with chronic myeloproliferative blood cancers are associated with an increased proportion of senescent T cells and signs of an aging immune system
Aging (Albany NY). 2021 Dec 26;13(undefined). doi: 10.18632/aging.203803. Online ahead of print.ABSTRACTThe cause of age-related macular degeneration (AMD) is unknown, but evidence indicates that both innate and adaptive immunity play a role in the pathogenesis. Our recent work has investigated AMD in patients with myeloproliferative neoplasms (MPNs) since they have increased drusen and AMD prevalence. We have previously found increased levels of chronic low-grade inflammation (CLI) in MPN patients with drusen (MPNd) compared to MPN patients with normal retinas (MPNn). CLI and AMD are both associated with aging, and we, th...
Source: Aging - December 26, 2021 Category: Biomedical Science Authors: Charlotte Liisborg Vibe Skov Lasse Kj ær Hans Carl Hasselbalch Torben Lykke S ørensen Source Type: research

Retinal drusen in patients with chronic myeloproliferative blood cancers are associated with an increased proportion of senescent T cells and signs of an aging immune system
Aging (Albany NY). 2021 Dec 26;13(undefined). doi: 10.18632/aging.203803. Online ahead of print.ABSTRACTThe cause of age-related macular degeneration (AMD) is unknown, but evidence indicates that both innate and adaptive immunity play a role in the pathogenesis. Our recent work has investigated AMD in patients with myeloproliferative neoplasms (MPNs) since they have increased drusen and AMD prevalence. We have previously found increased levels of chronic low-grade inflammation (CLI) in MPN patients with drusen (MPNd) compared to MPN patients with normal retinas (MPNn). CLI and AMD are both associated with aging, and we, th...
Source: Aging - December 26, 2021 Category: Biomedical Science Authors: Charlotte Liisborg Vibe Skov Lasse Kj ær Hans Carl Hasselbalch Torben Lykke S ørensen Source Type: research

IGFBP-6/sonic hedgehog/TLR4 signalling axis drives bone marrow fibrotic transformation in primary myelofibrosis
In conclusion, our results suggest that the IGFBP-6/SHH/TLR4 axis is implicated in alterations of the primary myelofibrosis microenvironment and that IGFBP-6 may play a central role in activating SHH pathway during the fibrotic process.PMID:34905501 | DOI:10.18632/aging.203779 (Source: Aging)
Source: Aging - December 14, 2021 Category: Biomedical Science Authors: Lucia Longhitano Daniele Tibullo Nunzio Vicario Cesarina Giallongo Enrico La Spina Alessandra Romano Sofia Lombardo Marina Moretti Francesco Masia Anna Rita Daniela Coda Santina Venuto Paolo Fontana Rosalba Parenti Giovanni Li Volti Michelino Di Rosa Gius Source Type: research

IGFBP-6/sonic hedgehog/TLR4 signalling axis drives bone marrow fibrotic transformation in primary myelofibrosis
In conclusion, our results suggest that the IGFBP-6/SHH/TLR4 axis is implicated in alterations of the primary myelofibrosis microenvironment and that IGFBP-6 may play a central role in activating SHH pathway during the fibrotic process.PMID:34905501 | DOI:10.18632/aging.203779 (Source: Aging)
Source: Aging - December 14, 2021 Category: Biomedical Science Authors: Lucia Longhitano Daniele Tibullo Nunzio Vicario Cesarina Giallongo Enrico La Spina Alessandra Romano Sofia Lombardo Marina Moretti Francesco Masia Anna Rita Daniela Coda Santina Venuto Paolo Fontana Rosalba Parenti Giovanni Li Volti Michelino Di Rosa Gius Source Type: research

IGFBP-6/sonic hedgehog/TLR4 signalling axis drives bone marrow fibrotic transformation in primary myelofibrosis
In conclusion, our results suggest that the IGFBP-6/SHH/TLR4 axis is implicated in alterations of the primary myelofibrosis microenvironment and that IGFBP-6 may play a central role in activating SHH pathway during the fibrotic process.PMID:34905501 | DOI:10.18632/aging.203779 (Source: Aging)
Source: Aging - December 14, 2021 Category: Biomedical Science Authors: Lucia Longhitano Daniele Tibullo Nunzio Vicario Cesarina Giallongo Enrico La Spina Alessandra Romano Sofia Lombardo Marina Moretti Francesco Masia Anna Rita Daniela Coda Santina Venuto Paolo Fontana Rosalba Parenti Giovanni Li Volti Michelino Di Rosa Gius Source Type: research

Tmprss6-ASO as a tool for the treatment of Polycythemia Vera mice
PLoS One. 2021 Dec 10;16(12):e0251995. doi: 10.1371/journal.pone.0251995. eCollection 2021.ABSTRACTPolycythemia Vera (PV) is a chronic myeloproliferative neoplasm resulting from an acquired driver mutation in the JAK2 gene of hematopoietic stem and progenitor cells resulting in the overproduction of mature erythrocytes and abnormally high hematocrit, in turn leading to thromboembolic complications. Therapeutic phlebotomy is the most common treatment to reduce the hematocrit levels and consequently decrease thromboembolic risk. Here we demonstrate that, by using the iron restrictive properties of the antisense oligonucleoti...
Source: Cell Research - December 10, 2021 Category: Cytology Authors: Carla Casu Alison Liu Gianluca De Rosa Audrey Low Aae Suzuki Sayantani Sinha Yelena Z Ginzburg Charles Abrams Mariam Aghajan Shuling Guo Stefano Rivella Source Type: research

Polycythemia vera in pregnancy represents a challenge for a multidisciplinary collaboration: A case report and literature review
Exp Ther Med. 2022 Jan;23(1):19. doi: 10.3892/etm.2021.10941. Epub 2021 Nov 1.ABSTRACTPolycythemia vera (PV) is a rare chronic myeloproliferative neoplasm which represents an additional thrombotic factor in pregnancy. PV may be difficult to diagnose, particularly as its incidence is extremely uncommon among young women. The main diagnostic method involves a bone marrow biopsy, and high hemoglobin and platelet counts are usually indicative of the condition, after excluding other more frequent pathologies. PV is associated with a high risk of thrombosis, particularly in pregnancy, and requires anti-platelet treatment. At pre...
Source: Experimental and Therapeutic Medicine - November 24, 2021 Category: General Medicine Authors: Roxana Elena Bohiltea Emilia Niculescu-Mizil Bianca Margareta Mihai Florentina Furtunescu Ionita Ducu Octavian Munteanu Tiberiu Augustin Georgescu Corina Grigoriu Source Type: research

Immune Phenomena in Myeloid Neoplasms: An “Egg or Chicken” Question
Immune phenomena are increasingly reported in myeloid neoplasms, and include autoimmune cytopenias/diseases and immunodeficiency, either preceding or complicating acute myeloid leukemia, myelodysplastic syndromes (MDS), chronic myeloproliferative neoplasms, and bone marrow failure (BMF) syndromes. Autoimmunity and immunodeficiency are the two faces of a dysregulated immune tolerance and surveillance and may result, along with contributing environmental and genetic factors, in an increased incidence of both tumors and infections. The latter may fuel both autoimmunity and immune activation, triggering a vicious circle among ...
Source: Frontiers in Immunology - September 29, 2021 Category: Allergy & Immunology Source Type: research

Cancers, Vol. 13, Pages 4750: Philadelphia-Negative Chronic Myeloproliferative Neoplasms during the COVID-19 Pandemic: Challenges and Future Scenarios
samonti An outbreak of severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) started in December 2019 in China and then become pandemic in February 2020. Several publications investigated the possible increased rate of COVID-19 infection in hematological malignancies. Based on the published data, strategies for the management of chronic Philadelphia-negative chronic myeloproliferative neoplasms (MPNs) are provided. The risk of severe COVID-19 seems high in MPN, particularly in patients with essential thrombocythemia, but not negligible in myelofibrosis. MPN patients are at high risk of both thrombotic and hemorrh...
Source: Cancers - September 23, 2021 Category: Cancer & Oncology Authors: Francesca Palandri Massimo Breccia Valerio De Stefano Francesco Passamonti Tags: Review Source Type: research

The JAK –STAT pathway: an emerging target for cardiovascular disease in rheumatoid arthritis and myeloproliferative neoplasms
AbstractInflammation contributes centrally to cardiovascular diseases, and anti-inflammatory treatments can reduce cardiovascular events. The JAK –STAT pathway is an emerging target in inflammation, mainly in rheumatoid arthritis (RA) and chronic myeloproliferative neoplasms (MPNs), disorders that heighten cardiovascular risk. The aim of this study was to review the international literature on the relationship between dysregulation of the J AK–STAT pathway in RA/MPNs and cardiovascular risk and on the potential cardiovascular effects of JAK–STAT inhibitors. The JAK–STAT pathway sustains inflammatory and thrombotic ...
Source: European Heart Journal - August 3, 2021 Category: Cardiology Source Type: research

Real-World Dosing Patterns of Ruxolitinib in Patients With Polycythemia Vera Who Are Resistant to or Intolerant of Hydroxyurea
Polycythemia vera (PV) is a Philadelphia chromosome-negative chronic myeloproliferative neoplasm affecting more than 100,000 people in the United States.1 The disease is characterized by an abnormal increase in red cell mass and the presence of JAK2 V617F or JAK2 exon 12 mutations.2 Patients with PV are at risk for thromboembolic events (TEs), premature death, and burdensome symptoms.3-5 The goals of PV therapy are to prevent TEs and hemorrhagic complications and to manage disease-related symptoms. (Source: Clinical Lymphoma, Myeloma and Leukemia)
Source: Clinical Lymphoma, Myeloma and Leukemia - July 4, 2021 Category: Hematology Authors: Ivy Altomare, Shreekant Parasuraman, Dilan Paranagama, Jonathan Kish, Kevin Lord, Jingbo Yu, Philomena Colucci Source Type: research