Chronic Myeloproliferative Neoplasm Research 
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Authors: Genthon A, Killian M, Mertz P, Cathebras P, Gimenez De Mestral S, Guyotat D, Chalayer E
Abstract
Myelofibrosis is a BCR-ABL1-negative chronic myeloproliferative neoplasm that includes primary myelofibrosis, post-polycythemia vera myelofibrosis, and post-essential thrombocythemia myelofibrosis. It is characterized by stem cell-derived clonal proliferation that is often, but not always, accompanied by somatic mutations, which are classified into driver mutations (JAK2, CALR, or MPL), subclonal mutations and fibrosis on bone marrow biopsy. Myelofibrosis commonly demonstrates splenomegaly, constitutio...
Source: Revue de Medecine Interne - November 29, 2020 Category: Internal Medicine Tags: Rev Med Interne Source Type: research
Current clinical strategies and emergent treatment landscapes in leukemic transformation of Philadelphia-negative myeloproliferative neoplasms.
Authors: Abruzzese E, Niscola P
Abstract
INTRODUCTION: Transformation to acute myeloid leukemia (AML) of Philadelphia chromosome-negative (Ph-) chronic myeloproliferative neoplasms (MPN) represents a challenging medical concern and an unmet clinical need, since it charts a very poor outcome and a low rate of response to standard treatments with the exception of allogeneic hematopoietic stem cell transplantation (HSCT). Recent novel insights into the molecular disease pathways and the genomic features characterizing the transformation of Ph-MPN have led to new therapeutic individualized approaches with the ...
Source: Expert Review of Hematology - November 25, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research
Austrian recommendations for the management of essential thrombocythemia.
Abstract
According to the World Health Organization (WHO) classification, essential (primary) thrombocythemia (ET) is one of several Bcr-Abl negative chronic myeloproliferative neoplasms (MPN). The classical term MPN covers the subcategories of MPN: ET, polycythemia vera (PV), primary myelofibrosis (PMF), and prefibrotic PMF (pPMF). ET is marked by clonal proliferation of hematopoietic stem cells, leading to a chronic overproduction of platelets. At the molecular level a JAK2 (Janus Kinase 2), calreticulin, or MPL mutation is found in the majority of patients. Typical ongoing complications of the disease...
Source: Wiener Klinische Wochenschrift - November 19, 2020 Category: General Medicine Authors: Buxhofer-Ausch V, Heibl S, Sliwa T, Beham-Schmid C, Wolf D, Geissler K, Krauth MT, Krippl P, Petzer A, Wölfler A, Melchardt T, Gisslinger H Tags: Wien Klin Wochenschr Source Type: research
Cancers, Vol. 12, Pages 3061: Risks of Solid and Lymphoid Malignancies in Patients with Myeloproliferative Neoplasms: Clinical Implications
sen
In the past decade, several studies have reported that patients with chronic myeloproliferative neoplasms (MPNs) have an increased risk of second solid cancer or lymphoid hematological cancer. In this qualitative review study, we present results from studies that report on these cancer risks in comparison to cancer incidences in the general population or a control group. Our literature search identified 12 such studies published in the period 2009–2018 including analysis of more than 65,000 patients. The results showed that risk of solid cancer is 1.5- to 3.0-fold elevated and the risk of lymphoid hem...
Source: Cancers - October 20, 2020 Category: Cancer & Oncology Authors: Mette Brabrand Henrik Frederiksen Tags: Review Source Type: research
Is there a gender effect in polycythemia vera?
AbstractIn recent times, there has been a growing interest in understanding the impact of gender on disease biology and clinical outcomes in Philadelphia-negative chronic myeloproliferative neoplasms. Among those, polycythemia vera (PV) is characterized by increased thrombotic risk, systemic symptoms, and overall reduced survival. Here, we aim to summarize data on whether and to what extent female sex can affect PV biology and outcome. To this end, we will discuss the latest acquisitions in terms of pathogenesis, diagnosis, epidemiology, clinical presentation and symptoms burden, thrombotic risk and related treatment strat...
Source: Annals of Hematology - October 1, 2020 Category: Hematology Source Type: research
Cancers, Vol. 12, Pages 2700: Risk of Inflammatory Bowel Disease in Patients with Chronic Myeloproliferative Neoplasms: A Danish Nationwide Cohort Study
Frederiksen
An association between hematological cancers and inflammatory bowel disease (IBD) has previously been suggested, but the risk of IBD in patients with myeloproliferative neoplasms (MPNs) is unknown. We conducted a nationwide population-based cohort study using Danish registries, to estimate the risk of IBD in individuals diagnosed with essential thrombocythemia, polycythemia vera, myelofibrosis or unclassifiable MPN during 1994–2013. MPN patients were matched 1:10 with sex- and age-matched comparisons. Everyone was followed until a diagnosis of IBD, death/emigration, or 31 December 2013. The ri...
Source: Cancers - September 20, 2020 Category: Cancer & Oncology Authors: Marie Bak Tine Jess Esben Meulengracht Flachs Ann-Dorthe Zwisler Knud Juel Henrik Frederiksen Tags: Article Source Type: research
BCR-ABL1–Negative Chronic Myeloproliferative Neoplasms and Pulmonary Hypertension: A Prospective Long-Term Follow-up Study of the Impact of Pulmonary Hypertension on Survival
Publication date: Available online 18 August 2020Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Kadriye Bahriye Payzin, Kaan Savasoglu, Inci Alacacioglu, Elif Ebru Dalgic, Betul Bolat Kucukzeybek, Aylin Orgen Calli, Sadi Bener, Serdar Payzin (Source: Clinical Lymphoma Myeloma and Leukemia)
Source: Clinical Lymphoma Myeloma and Leukemia - September 11, 2020 Category: Cancer & Oncology Source Type: research
Non-Pharmacologic Management of Splenomegaly for Patients with Myelofibrosis: Is There Any Role for Splenectomy or Splenic Radiation in 2020?
AbstractPurpose of ReviewMyelofibrosis is a chronic myeloproliferative neoplasm which can lead to massive splenomegaly. Currently approved medical therapies do not improve splenomegaly in all patients and effects are not sustained. Thus, spleen-directed therapies (i.e., splenectomy and splenic irradiation) have been used in some cases to palliate the signs and symptoms of the disease. Here, we critically review the literature regarding palliative splenectomy and splenic irradiation in myelofibrosis, and discuss their position in the current treatment landscape.Recent FindingsRetrospective studies have demonstrated that spl...
Source: Current Hematologic Malignancy Reports - August 21, 2020 Category: Hematology Source Type: research
BCR-ABL1 Negative Chronic Myeloproliferative Neoplasms and Pulmonary Hypertension: A Prospective Long Term Follow up Study of the impact of Pulmonary Hypertension on Survival
Publication date: Available online 18 August 2020Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Kadriye Bahriye Payzin, Kaan Savasoglu, Inci Alacacioglu, Elif Ebru Dalgic, Betul Bolat Kucukzeybek, Aylin Orgen Calli, Sadi Bener, Serdar Payzin (Source: Clinical Lymphoma Myeloma and Leukemia)
Source: Clinical Lymphoma Myeloma and Leukemia - August 18, 2020 Category: Cancer & Oncology Source Type: research
BCR-ABL1 –Negative Chronic Myeloproliferative Neoplasms and Pulmonary Hypertension: A Prospective Long-Term Follow-up Study of the Impact of Pulmonary Hypertension on Survival
This prospective study assessed the prevalence of pulmonary hypertension (PHT) related to chronic myeloproliferative neoplasms (CMPNs) and evaluated the impact of PHT on survival during long-term follow-up. In a large group of BCR-ABL1 –negative CMPN patients, there was a low prevalence of PHT. The impact of PHT on survival was negligible. (Source: Clinical Lymphoma, Myeloma and Leukemia)
Source: Clinical Lymphoma, Myeloma and Leukemia - August 16, 2020 Category: Hematology Authors: Kadriye Bahriye Payzin, Kaan Savasoglu, Inci Alacacioglu, Elif Ebru Dalgic, Betul Bolat Kucukzeybek, Aylin Orgen Calli, Sadi Bener, Serdar Payzin Tags: Original Study Source Type: research
BCR-ABL1 Negative Chronic Myeloproliferative Neoplasms and Pulmonary Hypertension: A Prospective Long Term Follow up Study of the impact of Pulmonary Hypertension on Survival
In this prospective study, we aimed to assess the prevalence of PHT in patients with BCR-ABL1 negative CMPN, and to evaluate impact of PHT on survival during long term follow-up. (Source: Clinical Lymphoma, Myeloma and Leukemia)
Source: Clinical Lymphoma, Myeloma and Leukemia - August 16, 2020 Category: Hematology Authors: Kadriye Bahriye Payzin, Kaan Savasoglu, Inci Alacacioglu, Elif Ebru Dalgic, Betul Bolat Kucukzeybek, Aylin Orgen Calli, Sadi Bener, Serdar Payzin Tags: Original Study Source Type: research
Hemorrhagic stroke associated with essential thrombocythemia: Case report and literature review
Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm characterized by the overproduction of circulating platelets in the peripheral blood, caused by the excessive proliferation of megakaryocytes in the bone marrow.1 Cerebrovascular events, mainly ischemic stroke, are one of the most serious complications in patients with ET, and the V617F mutation in the Janus kinase 2 (JAK2) gene is recognized as a thrombotic risk factor.2 Hemorrhagic stroke associated with ET is very infrequent, and the effect of mutation types on hemorrhagic stroke remains unclear. (Source: Journal of Stroke and Cerebrovascular Diseases)
Source: Journal of Stroke and Cerebrovascular Diseases - August 11, 2020 Category: Neurology Authors: Akihiko Momozaki, Jun Masuoka, Takashi Furukawa, Motofumi Koguchi, Hiroshi Ito, Fumitaka Yoshioka, Kohei Inoue, Atsushi Ogata, Yukiko Nakahara, Tatsuya Abe Tags: Case Report Source Type: research
Predicting Survival after Allogeneic Hematopoietic Cell Transplantation in Myelofibrosis: Performance of the Myelofibrosis Transplant Scoring System (MTSS) and Development of a New Prognostic Model
Myelofibrosis (MF) is a chronic myeloproliferative neoplasm (MPN) with a highly heterogeneous but potentially aggressive clinical course. Median survival is around 6 years1. Allogeneic hematopoietic cell transplantation (allo-HCT) remains the only curative treatment, yet the advanced age of MF patients and significant transplant-related mortality limit the applicability of this procedure2. (Source: Biology of Blood and Marrow Transplantation)
Source: Biology of Blood and Marrow Transplantation - July 23, 2020 Category: Hematology Authors: Juan-Carlos Hern ández-Boluda, Arturo Pereira, Alberto Alvarez-Larran, Ana-Africa Martín, Ana Benzaquen, Lourdes Aguirre, Elvira Mora, Pedro González, Jorge Mora, Nieves Dorado, Antonia Sampol, Valentín García-Gutiérrez, Oriana López-Godino, María Source Type: research
Cancers, Vol. 12, Pages 1763: Cancer Immune Therapy for Philadelphia Chromosome-Negative Chronic Myeloproliferative Neoplasms
rsen
Philadelphia chromosome-negative chronic myeloproliferative neoplasms (MPN) are neoplastic diseases of the hematopoietic stem cells in the bone marrow. MPN are characterized by chronic inflammation and immune dysregulation. Of interest, the potent immunostimulatory cytokine interferon-α has been used to treat MPN for decades. A deeper understanding of the anti-cancer immune response and of the different immune regulatory mechanisms in patients with MPN has paved the way for an increased perception of the potential of cancer immunotherapy in MPN. Therapeutic vaccination targeting the driver mutations ...
Source: Cancers - July 1, 2020 Category: Cancer & Oncology Authors: Morten Orebo Holmstr öm Hans Carl Hasselbalch Mads Hald Andersen Tags: Review Source Type: research
Modern management of splenomegaly in patients with myelofibrosis
AbstractMyelofibrosis (MF) is a chronic myeloproliferative neoplasm which can lead to massive splenomegaly secondary to extramedullary hematopoiesis. Patients frequently exhibit debilitating symptoms including pain and early satiety, in addition to cellular sequestration causing severe cytopenias. JAK 1/2 inhibitors, such as ruxolitinib and fedratinib, are the mainstay of therapy and produce significant and durable reductions in spleen volume. However, many patients are not eligible for JAK 2 inhibitor therapy or become refractory to treatment over time. Novel therapies are in development that can reduce the degree of sple...
Source: Annals of Hematology - May 16, 2020 Category: Hematology Source Type: research
