7,919 results match your criteria Agnogenic Myeloid Metaplasia With Myelofibrosis


[Clinical and epidemiological characteristics of the Philadelphia negative myeloproliferative neoplasms in Chile].

Rev Med Chil 2021 Dec;149(12):1687-1693

Hospital del Salvador, Santiago, Chile.

Background: Philadelphia-negative myeloproliferative neoplasms (Ph-MPN) are chronic hematological disorders characterized by the overproduction of one or more mature myeloid blood cell lineages. Classical Ph-MPN are polycythemia vera (PV), essential thrombocytopenia (ET) and primary myelofibrosis (PMF).

Aim: To assess the epidemiological, clinical and diagnostic characteristics of Ph-MPN in Chile. Read More

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December 2021

[Clinical and epidemiological characteristics of the Philadelphia negative myeloproliferative neoplasms in Chile].

Rev Med Chil 2021 Nov;149(11):1532-1538

Hospital del Salvador, Santiago, Chile.

Background: Philadelphia-negative myeloproliferative neoplasms (Ph-MPN) are chronic hematological disorders characterized by the overproduction of one or more mature myeloid blood cell lineages. Classical Ph-MPN are polycythemia vera (PV), essential thrombocytopenia (ET) and primary myelofibrosis (PMF).

Aim: To assess the epidemiological, clinical and diagnostic characteristics of Ph-MPN in Chile. Read More

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November 2021

Clinical features and next-generation sequencing landscape of essential thrombocythemia, prefibrotic primary myelofibrosis, and overt fibrotic primary myelofibrosis: a Chinese monocentric retrospective study.

J Cancer Res Clin Oncol 2022 Jun 22. Epub 2022 Jun 22.

Department of Hematology, The Fourth Affiliated Hospital of Zhejiang University School of Medicine, N1 Shangcheng Road, Yiwu, Zhejiang, People's Republic of China.

Objective: Since prefibrotic primary myelofibrosis (pre-PMF) was recognized as a separate entity in the 2016 revised classification of MPN differed from essential thrombocythemia (ET) or overt fibrotic primary myelofibrosis (overt PMF), it has been a subject of debate among experts due to its indefinite diagnosis.

Methods: We retrospectively reviewed the clinical parameters, haematologic information, and genetic mutations of patients who were diagnosed with myeloproliferative neoplasms (MPNs) according to the WHO 2016 criteria in China, including 56 ET patients, 19 pre-PMF patients, and 43 overt PMF patients.

Results: Pre-PMF patients exhibited higher leukocyte counts [14. Read More

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Lower CXCR3 expression in both patients with neovascular AMD and advanced stages of chronic myeloproliferative blood cancers.

PLoS One 2022 16;17(6):e0269960. Epub 2022 Jun 16.

Department of Ophthalmology, Zealand University Hospital, Roskilde, Denmark.

Purpose: Peripheral T cell CXCR3 expression has been found uniquely lower in patients having neovascular age-related macular degeneration (nAMD) than in healthy individuals. The CXCR3-axis has been shown to have angiostatic and antifibrotic properties. We have recently investigated systemic markers in patients with myeloproliferative neoplasms (MPNs) because of their higher prevalence of AMD, and we have observed higher systemic chronic low-grade inflammation and immunosenescence signs in MPNs with drusen (MPNd) compared to those with normal retinas (MPNn). Read More

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Recent knowledge of NFATc4 in oncogenesis and cancer prognosis.

Cancer Cell Int 2022 Jun 13;22(1):212. Epub 2022 Jun 13.

Laboratory of Cancer Biology and Epigenetics, Department of Cell Biology and Genetics, Shantou University Medical College, 22 Xinling Road, Shantou, Guangdong, 515041, People's Republic of China.

Nuclear factor of activated T-cells, cytoplasmic 4 (NFATc4), a transcription factor of NFAT family, which is activated by Ca/calcineurin signaling. Recently, it is reported that aberrantly activated NFATc4 participated and modulated in the initiation, proliferation, invasion, and metastasis of various cancers (including cancers of the lung, breast, ovary, cervix, skin, liver, pancreas, as well as glioma, primary myelofibrosis and acute myelocytic leukemia). In this review, we cover the latest knowledge on NFATc4 expression pattern, post-translational modification, epigenetic regulation, transcriptional activity regulation and its downstream targets. Read More

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[Clinical Analysis of Gene Mutation and Vascular Events in Patients with BCR/ABL Negative Myeloproliferative Neoplasms].

Authors:
Bao-Dan Hu Lin Chen

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2022 Jun;30(3):813-818

Department of Hematology, The Second Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China,E-mail:

Objective: To explore the relationship between clinical features, peripheral blood cell count, coagulation function, gene mutation and hemorrhagic events and thrombotic events in essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis(PMF) patients.

Methods: Clinical data of 78 patients with ET, PV, and PMF who were admitted to the Second Affiliated Hospital of Chongqing Medical University between September 2019 and August 2020 were retrospectively analyzed. Information about sex, age, gene mutation, peripheral blood cell count, coagulation function, and hemorrhagic and thrombotic events was included, and the influence of these data on the occurrence of hemorrhagic and thrombotic events was estimated. Read More

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[Analysis of the effect of gene mutations on the efficacy of ruxolitinib in patients with myelofibrosis based on second-generation sequencing technology].

Zhonghua Xue Ye Xue Za Zhi 2022 Apr;43(4):323-329

Department of Hematology, Nan fang Hospital, Southern Medical University, Guangzhou 510515, China.

To assess the effect of gene mutations on the efficacy of ruxolitinib for treating myelofibrosis (MF) . We retrospectively analyzed the clinical data of 56 patients with MF treated with ruxolitinib from July 2017 to December 2020 and applied second-generation sequencing (NGS) technology to detect 127 hematologic tumor-related gene mutations. Additionally, we analyzed the relationship between mutated genes and the efficacy of ruxolitinib. Read More

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[Influencing factors of iron metabolism assessment in patients with myelodysplastic syndrome: A retrospective study].

Zhonghua Xue Ye Xue Za Zhi 2022 Apr;43(4):293-299

Sixth People's Hospital Affiliated to Shanghai Jiao Tong University, Shanghai 200233, China.

To analyze the influencing factors of iron metabolism assessment in patients with myelodysplastic syndrome. MRI and/or DECT were used to detect liver and cardiac iron content in 181 patients with MDS, among whom, 41 received regular iron chelation therapy during two examinations. The adjusted ferritin (ASF) , erythropoietin (EPO) , cardiac function, liver transaminase, hepatitis antibody, and peripheral blood T cell polarization were detected and the results of myelofibrosis, splenomegaly, and cyclosporine were collected and comparative analyzed in patients. Read More

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Extramedullary hematopoiesis in an inguinal lymph node: an unusual presentation of primary myelofibrosis.

World J Surg Oncol 2022 Jun 8;20(1):186. Epub 2022 Jun 8.

Department of Histopathology, Indus Hospital and Health Network, Karachi, 75190, Pakistan.

Background: Extramedullary hematopoiesis (EMH) is a proliferation of hematopoietic tissue outside of the bone marrow medullary space. It is a pathophysiologic response, more often associated with either a benign reactive hematological disease or a myeloproliferative neoplasm (MPN). Identification of EMH in adults is always pathologic. Read More

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Marked Hyperbilirubinemia Associated with Primary Myelofibrosis Responsive to Ruxolitinib.

Case Reports Hepatol 2022 28;2022:9630996. Epub 2022 May 28.

Department of Gastroenterology & Hepatology, University of California, San Francisco- Fresno, Fresno, CA, USA.

Primary myelofibrosis (PMF) is a chronic myeloproliferative disorder seen in older adults which can present both with hepatosplenomegaly as well as mild nonspecific liver enzyme abnormalities. Mild elevations in bilirubin can occasionally be seen due to both intravascular hemolysis as well as extramedullary hematopoiesis. Marked hyperbilirubinemia as a presenting sign of PMF progression, however, has not been reported. Read More

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Eltrombopag may induce bone marrow fibrosis in allogeneic hematopoietic stem cell transplant recipients with prolonged thrombocytopenia.

Leuk Res 2022 Jul 21;118:106870. Epub 2022 May 21.

Gazi University Faculty of Medicine, Department of Pathology, Ankara, Turkey. Electronic address:

Poor graft function (PGF) and secondary failure of platelet recovery (SFPR) are significant causes of transplant related morbidity and mortality. Although thrombopoietin receptor agonists (TPO-RA), particularly Eltrombopag (EPAG), have been reported to be efficacious in the treatment of prolonged thrombocytopenia, potential long term adverse effects remain to be elucidated. This retrospective study was performed to determine the efficacy and toxicity profile of TPO-RAs in allogeneic hematopoietic stem cell transplant (alloHCT) recipients. Read More

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Primary myelofibrosis with thrombophilia as first symptom combined with thalassemia and Gilbert syndrome: A case report.

World J Clin Cases 2022 May;10(13):4161-4170

Department of Hematology, Peking Union Medical College Hospital, Beijing 100730, China.

Background: A 46-year-old Han man first had sigmoid sinus and transverse sinus venous thrombosis at the age of 42. At the age of 44, he once again developed thrombosis. Genetic testing showed heterozygous SERPINC1 mutation, bone marrow biopsy showed fibrosis grade 1 (MF-1), and JAK2 V617F mutation was positive, accompanied by UGT1A1 mutation and β-thalassemia gene mutation. Read More

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Management of Myeloproliferative Neoplasms in the Molecular Era: From Research to Practice.

Am Soc Clin Oncol Educ Book 2022 Apr;42:1-19

Global Oncology Program and Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA.

The 1960 discovery of the Philadelphia chromosome in chronic myeloid leukemia (CML) marked the beginning of the modern genomic era of oncology. In the following years, the molecular underpinnings of CML were unraveled, culminating in the development of the first molecularly targeted therapy: imatinib. Imatinib revolutionized CML management, inducing deep molecular responses for most patients and aligning survival curves with those of age-matched control participants. Read More

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Ruxolitinib withdrawal complicating emergency aortic root replacement.

Interact Cardiovasc Thorac Surg 2022 Jun;35(1)

Department of Cardiothoracic Surgery, Flinders Medical Centre, Adelaide, SA, Australia.

Ruxolitinib, a Janus kinase inhibitor, is associated with severe withdrawal phenomena. Adequate tapering is often underemphasized in surgical emergencies and can complicate the postoperative course. We present a case of acute ruxolitinib withdrawal in a gentleman undergoing emergency cardiac surgery. Read More

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In utero origin of myelofibrosis presenting in adult monozygotic twins.

Nat Med 2022 Jun 30;28(6):1207-1211. Epub 2022 May 30.

Medical Research Council (MRC) Molecular Haematology Unit, MRC Weatherall Institute of Molecular Medicine, National Institute for Health Research Biomedical Research Centre, University of Oxford, Oxford, UK.

The latency between acquisition of an initiating somatic driver mutation by a single-cell and clinical presentation with cancer is largely unknown. We describe a remarkable case of monozygotic twins presenting with CALR mutation-positive myeloproliferative neoplasms (MPNs) (aged 37 and 38 years), with a clinical phenotype of primary myelofibrosis. The CALR mutation was absent in T cells and dermal fibroblasts, confirming somatic acquisition. Read More

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Study of CALR, MPL, and c-kit Gene Mutations in Thai Patients with JAK2 V617F Negative Myeloproliferative Neoplasms.

Asian Pac J Cancer Prev 2022 May 1;23(5):1671-1678. Epub 2022 May 1.

Centre of Learning and Research in Celebration of HRH Princess Chulabhorn's 60th Birthday Anniversary, Chulabhorn Royal Academy, Bangkok, Thailand.

Objective: The aim of this study to determine the prevalence of CALR, MPL and c-kit gene mutations in JAK2 V617F negative-MPN patients.

Methods: The retrospective study of CALR, MPL and c-kit mutations were analyzed in 113 samples collected from March 2010 to May 2017 and identified as JAK2 V617F-negative MPN Thai patients. The samples were analysis by gel electrophoresis and direct sequencing. Read More

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The Odyssey of Pacritinib in Myelofibrosis.

Blood Adv 2022 May 27. Epub 2022 May 27.

Icahn School of Medicine at Mount Sinai, New York, New York, United States.

Myelofibrosis (MF) can present with symptomatic splenomegaly and/or cytopenias including thrombocytopenia. Disease related thrombocytopenia is a poor prognostic factor with a median overall survival of less than 2 years. Currently approved JAK1/2 inhibitors have not been evaluated in patients with platelets ≤50× 109/L, and in fact could potentiate thrombocytopenia due to their combined JAK1/2 inhibitory activity. Read More

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Primary myelofibrosis with concurrent paroxysmal nocturnal haemoglobinuria presenting with erectile dysfunction.

Oxf Med Case Reports 2022 May 23;2022(5):omac047. Epub 2022 May 23.

Departments of Medicine, Prince Saud Bin Jalawi Hospital, Eastern Province, Saudi Arabia.

Primary myelofibrosis and paroxysmal nocturnal haemoglobinuria (PNH) are uncommon clonal blood disorders that are rarely found together. We report a case of primary myelofibrosis (PMF) with concomitant subtle PNH in a 42-year-old man who presented with a 4-week history of fatigue, unexplained chest pain, and new-onset erectile dysfunction. Bone marrow biopsy showed severe fibrosis consistent with PMF. Read More

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Momelotinib for the treatment of myelofibrosis with anemia.

Future Oncol 2022 Jun 23;18(20):2559-2571. Epub 2022 May 23.

UT Health San Antonio Cancer Center, San Antonio, TX, USA.

Myelofibrosis is a myeloproliferative neoplasm characterized by splenomegaly, debilitating constitutional symptoms and bone marrow failure. Disease-related anemia is common and associated with an inferior quality of life and survival. Unfortunately, few therapies exist to improve hemoglobin in myelofibrosis patients. Read More

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Hippo pathway-related genes expression is deregulated in myeloproliferative neoplasms.

Med Oncol 2022 May 23;39(5):97. Epub 2022 May 23.

Laboratório de Fisiopatologia das Doenças Hematológicas, Departamento de Análises Clínicas, Toxicológicas e Bromatológicas, Faculdade de Ciências Farmacêuticas de Ribeirão Preto, Universidade de São Paulo - USP, Av. do Café, s/nº. Campus Universitário, Ribeirão Preto, SP, 14040-903, Brazil.

Myeloproliferative neoplasms (MPN) are hematological disorders characterized by increased proliferation of precursor and mature myeloid cells. MPN patients may present driver mutations in JAK2, MPL, and CALR genes, which are essential to describe the molecular mechanisms of MPN pathogenesis. Despite all the new knowledge on MPN pathogenesis, many questions remain to be answered to develop effective therapies to cure MPN or impair its progression to acute myeloid leukemia. Read More

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GLI1 activates pro-fibrotic pathways in myelofibrosis fibrocytes.

Cell Death Dis 2022 May 20;13(5):481. Epub 2022 May 20.

Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

Bone marrow (BM) fibrosis was thought to be induced exclusively by mesenchymal stromal cells (MSCs). However, we and others found that neoplastic fibrocytes induce BM fibrosis in myelofibrosis (MF). Because glioma-associated oncogene-1 (GLI1), an effector of the Hedgehog pathway, plays a role in the induction of BM fibrosis, we wondered whether GLI1 affects fibrocyte-induced BM fibrosis in MF. Read More

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How early intervention impacts long-term survival in myelofibrosis.

Authors:
Srdan Verstovsek

Clin Adv Hematol Oncol 2022 05;20(5):291-294

Hanns A. Pielenz Clinical Research Center for Myeloproliferative Neoplasms, Department of Leukemia, MD Anderson Cancer Center, Houston, Texas.

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Addition of navitoclax to ongoing ruxolitinib treatment in patients with myelofibrosis (REFINE): a post-hoc analysis of molecular biomarkers in a phase 2 study.

Lancet Haematol 2022 Jun 13;9(6):e434-e444. Epub 2022 May 13.

Guy's and St Thomas' NHS Foundation Trust, London, UK. Electronic address:

Background: Primary analyses of cohort 1a of the REFINE trial showed that addition of navitoclax to ruxolitinib induced a 35% or greater reduction in spleen volume (SVR) and reduced symptoms in patients with myelofibrosis no longer benefiting from ruxolitinib. Here, we report the exploratory post-hoc biomarker analyses from cohort 1a.

Methods: REFINE is a phase 2, multicentre, open-label trial designed to assess the activity and safety of navitoclax alone or in combination with ruxolitinib in patients with primary or secondary (post-polycythaemia vera or post-essential thrombocythaemia) myelofibrosis. Read More

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Making a case for disease-modifying agents in myelofibrosis.

Lancet Haematol 2022 06 13;9(6):e391-e392. Epub 2022 May 13.

Hematology Unit, ASST Sette Laghi, Ospedale di Circolo, Varese, Italy. Electronic address:

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Classic myeloproliferative neoplasms in Singapore: A population-based study on incidence, trends, and survival from 1968 to 2017.

Cancer Epidemiol 2022 May 13;79:102175. Epub 2022 May 13.

Department of Preventive and Population Medicine, Office of Clinical Epidemiology, Analytics, and Knowledge (OCEAN), Tan Tock Seng Hospital, Singapore. Electronic address:

Background: Current descriptive epidemiological information on classic myeloproliferative neoplasms (MPNs) is incomplete. Published data among Asian population are particularly sparse.

Methods: We conducted a large population-based study to determine the incidence rates and survival patterns of MPN reported to the Singapore Cancer Registry during the period 1968-2017. Read More

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Pacritinib: First Approval.

Authors:
Yvette N Lamb

Drugs 2022 May;82(7):831-838

Springer Nature, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.

Pacritinib (VONJO™) is an orally administered, small molecule kinase inhibitor being developed by CTI BioPharma for the treatment of myelofibrosis and graft-versus-host disease. Pacritinib received its first approval in February 2022 in the USA for the treatment of adults with intermediate- or high-risk primary or secondary (post-polycythemia vera or post-essential thrombocythemia) myelofibrosis with a platelet count below 50 × 10/L. The accelerated approval was based on results from the randomized, active-controlled, phase III PERSIST-2 trial, in which spleen volume reduction was demonstrated in pacritinib recipients. Read More

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Impact of Individual Comorbidities on Survival of Patients with Myelofibrosis.

Cancers (Basel) 2022 May 9;14(9). Epub 2022 May 9.

Hematology Department, Hospital Universitario Virgen de la Victoria, 29010 Málaga, Spain.

The comorbidity burden is an important risk factor for overall survival (OS) in several hematological malignancies. This observational prospective study was conducted to evaluate the impact of individual comorbidities on survival in a multicenter series of 668 patients with primary myelofibrosis (PMF) or MF secondary to polycythemia vera (PPV-MF) or essential thrombocythemia (PET-MF). Hypertension (hazard ratio (HR) = 4. Read More

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Primary autoimmune myelofibrosis: Twenty years in making.

Pediatr Blood Cancer 2022 May 13:e29762. Epub 2022 May 13.

Department of Hematology, All India Institute of Medical Sciences, New Delhi, India.

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RAPID LOW-COST DETECTION OF TYPE 2CALR MUTATION BY ALLELE-SPECIFIC RT-PCR FOR DIAGNOSIS OF MYELOPROLIFERATIVE NEOPLASMS.

Exp Oncol 2022 05;44(1):83-86

Institute of Molecular Biology and Genetics, National Academy of Sciences of Ukraine, Kyiv 03143, Ukraine.

Background: Approximately 15% to 24% of essential thrombocythemia (ET) and 25-35% of primary myelofibrosis cases carry a mutation in the calreticulin (CALR) gene. Sanger sequencing, qPCR, high resolution melt or targeted next generation sequencing usually used to detect these mutations are expensive and require costly equipment. Nevertheless, type 1 CALR mutations are detectable by using polymerase chain reaction (PCR) and agarose gel electrophoresis. Read More

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Splenic Infarction in Patients with Philadelphia-negative Myeloproliferative Neoplasms.

Intern Med 2022 May 7. Epub 2022 May 7.

Division of Hematology/Oncology, Department of Internal Medicine, Chungnam National University College of Medicine, Korea.

Objective We retrospectively analyzed the prevalence and clinical features of splenic infarctions in patients with Philadelphia chromosome-negative myeloproliferative neoplasms (Ph MPNs). Patients Patients diagnosed with essential thrombocythemia (ET), polycythemia vera (PV), prefibrotic/early primary myelofibrosis (pre-PMF), or PMF from January 1996 to October 2020 in Chungnam National University Hospital, Daejeon, Korea, were reviewed. Results A total of 347 patients (143 ET, 129 PV, 44 pre-PMF, and 31 PMF patients; 201 men and 146 women) with a median age of 64 (range 15-91) years old were followed up for a median of 4. Read More

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