Use the keywords feature with a free QxMD account.
Use Read by QxMD to access full text via your institution or open access sources.
Read also provides personalized recommendations to keep you up to date in your field.
Existing User
Sign InNew to Read
Sign Up#21
JOURNAL ARTICLE
Francesca Palandri, Elena Rossi, Giuseppe Auteri, Massimo Breccia, Simona Paglia, Giulia Benevolo, Elena M Elli, Francesco Cavazzini, Gianni Binotto, Alessia Tieghi, Mario Tiribelli, Florian H Heidel, Massimiliano Bonifacio, Novella Pugliese, Giovanni Caocci, Monica Crugnola, Francesco Mendicino, Alessandra D'Addio, Simona Tomassetti, Bruno Martino, Nicola Polverelli, Sara Ceglie, Camilla Mazzoni, Rikard Mullai, Alessia Ripamonti, Bruno Garibaldi, Fabrizio Pane, Antonio Cuneo, Mauro Krampera, Gianpietro Semenzato, Roberto M Lemoli, Nicola Vianelli, Giuseppe A Palumbo, Alessandro Andriani, Michele Cavo, Roberto Latagliata, Valerio De Stefano
In polycythemia vera (PV), the prognostic relevance of an ELN-defined complete response (CR) to hydroxyurea (HU), the predictors of response, and patients' triggers for switching to ruxolitinib are uncertain. In a real-world analysis, we evaluated the predictors of response, their impact on the clinical outcomes of CR to HU, and the correlations between partial or no response (PR/NR) and a patient switching to ruxolitinib. Among 563 PV patients receiving HU for ≥12 months, 166 (29.5%) achieved CR, 264 achieved PR, and 133 achieved NR...
July 21, 2023: Cancers
#22
Jin Zhang, Kefeng Shen, Min Xiao, Jinjin Huang, Jin Wang, Yaqin Wang, Zhenya Hong
Background: JAK2, CALR, and MPL gene mutations are recognized as driver mutations of myeloproliferative neoplasms (MPNs). MPNs without these mutations are called triple-negative (TN) MPNs. Recently, novel mutation loci were continuously discovered using next-generation sequencing (NGS), along with continued discussion and modification of the traditional TN MPN. Case presentation: Novel pathogenic mutations were discovered by targeted NGS in 4 patients who were diagnosed as JAK2 unmutated polycythaemia vera (PV) or TN MPN...
2023: Frontiers in Genetics
#23
REVIEW
Andrea Duminuco, Elena Torre, Giuseppe A Palumbo, Claire Harrison
PURPOSE OF REVIEW: Chronic myeloproliferative neoplasms (MPN) represent a group of diseases characterised by constitutive activation of the JAK/STAT pathway in a clonal myeloid precursor. The therapeutic approach aims to treat the symptom burden (headache, itching, debilitation), splenomegaly, slow down the fibrotic proliferation in the bone marrow and reduce the risk of thrombosis/bleeding whilst avoiding leukaemic transformation. RECENT FINDINGS: In recent years, the advent of JAK inhibitors (JAKi) has significantly broadened treatment options for these patients...
October 2023: Current Hematologic Malignancy Reports
#24
REVIEW
Claire Harrison, Ipek Cakmak
INTRODUCTION: Polycythemia vera (PV) is driven by mutations in JAK2 kinase and subsequent JAK/STAT activation, presentation can range from an asymptomatic state to micro or macrovascular events. Characteristic aquagenic pruritus and fatigue can have a substantial impact on quality of life. Over time, a minority will transform into more aggressive conditions such as post-PV myelofibrosis or acute myeloid leukemia. The JAK1 and 2 inhibitor Ruxolitinib has been approved for the treatment of PV after the failure of first-line therapies...
June 21, 2023: Expert Opinion on Pharmacotherapy
#25
REVIEW
Zi Yun Ng, Kathryn A Fuller, Allegra Mazza-Parton, Wendy N Erber
Myeloproliferative neoplasms (MPN) are a group of clonal haematological malignancies first described by Dameshek in 1957. The Philadelphia-negative MPN that will be described are polycythaemia vera (PV), essential thrombocythaemia (ET), pre-fibrotic myelofibrosis and primary myelofibrosis (PMF). The blood and bone marrow morphology are essential in diagnosis, for WHO classification, establishing a baseline, monitoring response to treatment and identifying changes that may indicate disease progression. The blood film changes may be in any of the cellular elements...
June 2023: International Journal of Laboratory Hematology
#26
JOURNAL ARTICLE
Eva Karadi, Peter Dombi, Viktoria Gyorine Korom, Eszter Kovacs, Jozsef Herczeg, Hajnalka Andrikovics, Arpad Illes, Judit Demeter, Lajos Homor, Miklós Udvardy, Miklós Egyed
OBJECTIVE: The Hungarian National Registry for Philadelphia chromosome negative myeloproliferative neoplasms was used to analyse the thromboembolic events (TE) of Hungarian patients with polycythemia vera (PV). METHODS: Data from 351 JAK2 V617F-positive patients diagnosed with PV were collected online from 15 haematology centres reporting clinical characteristics, therapeutic interventions and thromboembolic events. TE events were evaluated before and after diagnosis based upon the Landolfi and Tefferi risk assessment scales...
August 2023: European Journal of Haematology
#27
JOURNAL ARTICLE
Karen L Shires, Adriana J Rust, Rushil Harryparsad, Justin A Coburn, Ruth E Gopie
BACKGROUND: Mutations in JAK2/STAT5 proliferation pathway genes are key in the diagnosis of myeloproliferative neoplasms (MPNBCR/ABLneg ), with JAK2V617F being found in 50-97% of MPNBCR/ABLneg subtypes. Low JAK2V617F positivity at our facility suggested that our South African MPNBCR/ABLneg population may have a different mutational landscape. OBJECTIVES: We aimed to determine the JAK2/STAT5 mutation frequencies associated with our local MPNBCR/ABLneg population, thus determining the relevance of these molecular tests in this group...
April 4, 2023: Hematology/oncology and Stem Cell Therapy
#28
Catarina Negrão, Marta Machado, Margarida Mourato, Rita Sismeiro, Marta Jonet
Paraneoplastic pruritus has been reported mostly in association with haematological malignancies, and rarely with solid tumours. Aquagenic pruritus is itching without any skin lesion that develops a few minutes after contact with water of any temperature and it is associated with polycythaemia vera or other lymphoproliferative diseases. Here we report a case of a previously healthy 78-year-old Portuguese woman, who had been treated unsuccessfully for aquagenic pruritus for the previous eight months, and presented to the emergency department complaining of pain and swelling in her left leg...
February 2023: Curēus
#29
JOURNAL ARTICLE
Christelle Le Gall-Ianotto, Anne-Sophie Ficheux, Eric Lippert, Laura Herbreteau, Laetitia Rio, Brigitte Pan-Petesch, Laurent Misery, Jean-Christophe Ianotto
BACKGROUND: Pruritus is a frequent symptom experienced by patients with myeloproliferative neoplasms (MPN). Aquagenic pruritus (AP) is the most common type. The Myeloproliferative Neoplasm-Symptom Assessment Form Total Symptom Score (MPN-SAF TSS) self-report questionnaires were distributed to MPN patients before consultations. OBJECTIVES: The aim of this study was to assess clinical incidence (phenotypical evolution and response to treatment) of pruritus, especially AP, in MPN patients during their follow-ups...
February 21, 2023: Journal of the European Academy of Dermatology and Venereology: JEADV
#30
RANDOMIZED CONTROLLED TRIAL
Srdan Verstovsek, Aaron T Gerds, Alessandro M Vannucchi, Haifa Kathrin Al-Ali, David Lavie, Andrew T Kuykendall, Sebastian Grosicki, Alessandra Iurlo, Yeow Tee Goh, Mihaela C Lazaroiu, Miklos Egyed, Maria Laura Fox, Donal McLornan, Andrew Perkins, Sung-Soo Yoon, Vikas Gupta, Jean-Jacques Kiladjian, Nikki Granacher, Sung-Eun Lee, Luminita Ocroteala, Francesco Passamonti, Claire N Harrison, Barbara J Klencke, Sunhee Ro, Rafe Donahue, Jun Kawashima, Ruben Mesa
BACKGROUND: Janus kinase (JAK) inhibitors approved for myelofibrosis provide spleen and symptom improvements but do not meaningfully improve anaemia. Momelotinib, a first-in-class inhibitor of activin A receptor type 1 as well as JAK1 and JAK2, has shown symptom, spleen, and anaemia benefits in myelofibrosis. We aimed to confirm the differentiated clinical benefits of momelotinib versus the active comparator danazol in JAK-inhibitor-exposed, symptomatic patients with anaemia and intermediate-risk or high-risk myelofibrosis...
January 28, 2023: Lancet
#31
JOURNAL ARTICLE
Wenjing Gu, Jingyuan Tong, Rongfeng Fu, Ting Sun, Mankai Ju, Yanhong Zhao, Di Wang, Jie Gao, Jinhua Liu, Yuchen Gao, Huiyuan Li, Wentian Wang, Ying Chi, Renchi Yang, Lu Chen, Lihong Shi, Lei Zhang
JAK2V617F is the most frequent mutation in BCR-ABL-negative myeloproliferative neoplasms (MPNs). It is an important but not the only determinant of MPN phenotype. We performed high-throughput sequencing on JAK2V617F+ essential thrombocythaemia (ET) and polycythaemia vera (PV) patient samples to unveil factors involved in phenotypic heterogeneity and to identify novel therapeutic targets for MPN. Two concurrent mutations that may affect phenotype were identified, including mutations in SH2B3, which is primarily prevalent in PV, and SF3B1, which is more commonly mutated in ET...
January 28, 2023: British Journal of Haematology
#32
JOURNAL ARTICLE
Redaktion Facharztmagazine
No abstract text is available yet for this article.
February 2023: MMW Fortschritte der Medizin
#33
JOURNAL ARTICLE
Zoey Harris, Hannah Kaizer, Aria Wei, Theodoros Karantanos, Donna M Williams, Shruti Chaturvedi, Tania Jain, Linda Resar, Alison R Moliterno, Evan M Braunstein
The aim of this study was to compare the genomic features and clinical outcomes between paediatric and young adult patients (PAYA, <40 years) and older adults (OA, ≥40 years) with myeloproliferative neoplasms (MPN) to gain insight into pathogenesis, disease prognosis and management. Of 630 MPN patients, 171 (27%) were PAYA with an average age at diagnosis of 31 years. Females were more prevalent in PAYA than OA (71% vs 58%; p = 0.002), and PAYA more frequently presented with essential thrombocytosis (ET) at diagnosis (67% vs 39%; p < 0...
January 16, 2023: British Journal of Haematology
#34
REVIEW
Yuh Cai Chia, Mat Jusoh Siti Asmaa, Marini Ramli, Peng Yeong Woon, Muhammad Farid Johan, Rosline Hassan, Md Asiful Islam
Classical BCR-ABL -negative myeloproliferative neoplasms (MPN) include polycythaemia vera, essential thrombocythaemia, and primary myelofibrosis. Unlike monogenic disorders, a more complicated series of genetic mutations are believed to be responsible for MPN with various degrees of thromboembolic and bleeding complications. Thrombosis is one of the early manifestations in patients with MPN. To date, the driver genes responsible for MPN include JAK2 , CALR , MPL , TET2 , ASXL1 , and MTHFR . Affords have been done to elucidate these mutations and the incidence of thromboembolic events...
January 3, 2023: Diagnostics
#35
JOURNAL ARTICLE
Kyle Cilia, Janice Borg, Maria Bugeja, Emanuel Farrugia
We report a case of a man in his early 60s presenting with bilateral adrenal and pulmonary haemorrhages as an initial presentation of polycythaemia vera (PV). Symptomatology included severe compressive chest pain radiating to epigastrium, with unremarkable physical findings, parameters and ECG. Blood investigations showed an elevated haemoglobin (174 g/L, reference range (RR): 141-172g/L) and haematocrit (55.7%, RR: 40.4%-50.4%) levels.Cross-sectional imaging excluded aortic dissection, but imaging repeated 48 hours after his admission for acute dyspnoea and worsening abdominal pain showed bilateral alveolar and adrenal haemorrhages...
December 8, 2022: BMJ Case Reports
#36
Jumoke Okikiolu, Claire Woodley, Llywelyn Cadman-Davies, Jennifer O'Sullivan, Deepti Radia, Natalia Curto Garcia, Patrick Harrington, Shahram Kordasti, Susan Asirvatham, Priya Sriskandarajah, Jamie Saunders, Chandan Saha, Irene Sanchez, Hugues deLavallade, Donal P McLornan, Claire N Harrison
Despite widespread use of Pegylated forms of Inteferon in the management of Myeloproliferative Neoplasms (MPN), most clinicians have experience predominantly with peginterferon alfa-2a (Pegasys). Third generation pegylated IFNα, ropeginterferon alfa-2b (ropegIFN; Besremi), was recommended by the European Medicine Authority (EMA) for treatment of Polycythaemia Vera (PV) following a Phase III trial (PROUD-PV / CONTINUATION-PV). FDA approval for PV, regardless of treatment history, was subsequently granted in November 2021...
2023: Leukemia Research Reports
#37
JOURNAL ARTICLE
Ngoda Manongi, Kory Byrns, Malvina Fulman, Amir Jaffer
Inferior mesenteric vein thrombosis (IMVT) is a rare and potentially lethal complication that can progress to mesenteric ischaemia if left untreated. We report a case of a man in his 70s with a history of polycythaemia vera (PV) with a known negative Janus kinase-2 mutation ( JAK2V617 ) who was started on testosterone therapy 2 months before presenting with left lower quadrant abdominal and flank pain. Evaluation revealed thrombocytosis and IMVT on CT scan. A non-operative approach was deferred for anticoagulation therapy...
December 14, 2022: BMJ Case Reports
#38
JOURNAL ARTICLE
Abhinav Mathur, Joakim Edman, Livia Liang, Neil W Scott, Henry G Watson
Hydroxycarbamide (HC) is used as a cytoreductive treatment in myeloproliferative neoplasms (MPN). Observational studies have raised the possibility that HC contributes to the development of secondary malignancies, including skin tumours in MPN patients. In this retrospective observational study, we report a single-centre experience of 324 HC-treated MPN patients with long-term follow-up, compared to 47 MPN patients not on HC. Thirty-three patients (10.2%) (HC) versus one patient (2.1%) (no HC) developed skin tumours during follow-up (Hazard ratios [HR] 5...
November 2022: EJHaem
#39
JOURNAL ARTICLE
E B Yasin, R Alserihi, H Alkhatabi, H M H Qutob, R Qahwaji, S W Kattan, K A Gholam, E Hussein, A S Barefah, M E Alghuthami, A Abuzenadah
OBJECTIVE: Janus kinase/signal transducer and activator of transcription (JAK/STAT) pathway activation is initiated by mutations in the JAK2 gene. This activation is in turn, a vital pathogenetic mechanism in myeloproliferative neoplasms (MPNs). However, several factors affect the pathogenesis of MPNs other than the JAK2 gene mutations, such as the downregulation of cytokine signaling (SOCS) proteins, which are potent inhibitors of the JAK/STAT pathway. Therefore, we hypothesized that the regulation of SOCS protein system might be a possible pathogenetic mechanism of MPNs through activating the JAK/STAT pathway...
November 2022: European Review for Medical and Pharmacological Sciences
#40
JOURNAL ARTICLE
Ziqing Wang, Weiyi Liu, Dehao Wang, Erpeng Yang, Yujin Li, Yumeng Li, Yan Sun, Mingjing Wang, Yan Lv, Xiaomei Hu
Thrombosis is a common complication of myeloproliferative neoplasm (MPN), and it is a major cause of disability and death. With the development of next-generation gene-sequencing technology, the relationship between non-driver mutations and thrombotic risk factors has also attracted considerable attention. To analyze the risk factors of thrombosis in patients with essential thrombocythemia (ET) and polycythemia vera (PV), we retrospectively analyzed the clinical data of 125 MPN patients (75 ET and 50 PV) and performed a multivariate analysis of the risk factors of thrombosis using a Cox proportional risk model...
November 8, 2022: Journal of Clinical Medicine
Make the most of Read.
Download the mobile app.
Download the mobile app.
Fetching more papers... 
