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Semin Thromb Hemost 2021 Jun 11. Epub 2021 Jun 11.

Department of Medicine and Surgery, University of Insubria, Varese, Italy.

Splanchnic vein thrombosis (SVT), which includes portal, mesenteric, and splenic vein thrombosis and the Budd-Chiari syndrome, is an infrequent manifestation of venous thromboembolism (VTE). Like typical site VTE, SVT is also frequently associated with cancer, particularly intra-abdominal solid malignancies and myeloproliferative neoplasms (MPNs). The clinical presentation of SVT is nonspecific. Read More

BMC Cancer 2021 Jun 10;21(1):691. Epub 2021 Jun 10.

Department of Hematology and Medical Oncology, Mayo Clinic, 13400 East Shea Boulevard, Scottsdale, AZ, 85259, USA.

Background: Patients with Philadelphia-negative Myeloproliferative Neoplasms (MPN) suffer from numerous symptoms and decreased quality of life. Smoking is associated with an increased symptom burden in several malignancies. The aim of this study was to analyze the association between smoking and MPN-related symptom burden and explore MPN patients' opinions on smoking. Read More

J Vet Intern Med 2021 Jun 10. Epub 2021 Jun 10.

Department of Veterinary Medicine and Epidemiology, University of California, Davis, California, USA.

Primary erythrocytosis (PE) is a rare myeloproliferative neoplasm in cats resulting in the overproduction of erythrocytes. Current treatment modalities include repeated phlebotomy and chemotherapeutic drugs. These treatments may not be well tolerated by the cat and can present safety and financial challenges to owners. Read More

Rinsho Ketsueki 2021 ;62(5):346-351

Department of Hematology/Oncology, Saitama Children's Medical Center.

A 14-year-old male with autism was admitted to our hospital owing to altered consciousness and gait disturbance. Blood tests showed a white blood cell (WBC) count of 728,600/µl, and brain computed tomography revealed intracranial hemorrhage and a midline shift of the brain. The chronic phase of chronic myeloid leukemia (CML) was confirmed as per bone marrow aspiration findings. Read More

Klin Lab Diagn 2021 06;66(6):379-384

Krasnoyarsk branch of the «National Research Center for Hematology» Department of Health.

Overactive JAK pathway signaling is a hallmark of immune diseases and critically affects on inflammation and coagulation. A number of mutations in the JAK2 gene act as driving forces of myeloproliferative neoplasms (MPN), the pathogenesis of certain variants of acute leukemia, a number of solid malignancies and cardiovascular diseases. Assays for quantifying JAK2 mRNA in circulating blood cells can be used as a marker associated with the activity of this enzyme. Read More

Anticancer Agents Med Chem 2021 Jun 8. Epub 2021 Jun 8.

Key Laboratory of Marine Drugs, The Ministry of Education of China, School of Medicine and Pharmacy, Ocean University of China, Qingdao 266003, China.

Background: Chronic myeloid leukemia (CML) is a myeloproliferative disease caused by BCR-ABL oncoprotein. Tyrosine kinase inhibitors have been developed to inhibit the activity of BCR-ABL; however, drug resistance and side effect occur in clinic application. Therefore, it is urgent to find novel drugs for CML treatment. Read More

Curr Treat Options Oncol 2021 Jun 7;22(7):59. Epub 2021 Jun 7.

Department of Internal Medicine, Division of Hematology, Mayo Clinic, 200 First St. SW, Rochester, MN, 55905, USA.

Opinion Statement: Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative neoplasm (MPN) characterized by oncogenic driver mutations in colony-stimulating factor 3 receptor (CSF3R). Due in large part to the rarity of the disease and dearth of clinical trials, there is currently no standard of care for CNL. Available therapies range from conventional oral chemotherapy to targeted JAK inhibitors to hematopoietic stem cell transplant (HSCT), the latter representing the only potentially curative modality. Read More

Hemasphere 2021 Jun 1;5(6):e586. Epub 2021 Jun 1.

Department of Hematology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, the Netherlands.

Acquired hemophilia A and acquired von Willebrand syndrome are rare, but life-threatening bleeding disorders that require prompt diagnosis and treatment by hematologists. Acquired hemophilia A is defined as an acquired severe bleeding tendency caused by autoantibody formation against coagulation factor VIII. Acquired von Willebrand syndrome is characterized by a new onset bleeding tendency caused by a reduced concentration and/or function of von Willebrand factor. Read More

J Environ Anal Toxicol 2021 Feb;8(1)

Icahn School of Medicine, Mt. Sinai, New York, NY, USA.

In 2006, the Agency for Toxic Substances and Disease Registry received a request to determine whether a cluster of polycythemia vera patients existed in a northeast Pennsylvania community. A significant cluster of PV cases was identified at the nexus of three counties near several hazardous waste sites. The current study evaluated the potential for a select number of environmental contaminants previously detected in the cluster area to induce DNA damage using assays with hematopoietic stem-cell derived progenitor cells. Read More

Front Oncol 2021 18;11:665037. Epub 2021 May 18.

Department of Clinical Analyses, Toxicology and Food Science, School of Pharmaceutical Sciences of Ribeirão Preto, University of São Paulo, Ribeirão Preto, Brazil.

Background: Essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis (PMF) are clonal hematological diseases classified as Philadelphia chromosome-negative myeloproliferative neoplasms (MPN). MPN pathogenesis is associated with the presence of somatic driver mutations, bone marrow (BM) niche alterations, and tumor inflammatory status. The relevance of soluble mediators in the pathogenesis of MPN led us to analyze the levels of cytokines, chemokines, and growth factors related to inflammation, angiogenesis and hematopoiesis regulation in the BM niche of MPN patients. Read More

Clin Exp Allergy 2021 Jun 3. Epub 2021 Jun 3.

Department of Respiratory Sciences, College of Life Sciences, Institute for Lung Health, NIHR Leicester Biomedical Research Centre (Respiratory theme), University of Leicester and Respiratory and Allergy Services, University Hospitals of Leicester NHS Trust, Leicester, UK.

Background: A peripheral blood eosinophilia of greater than 1.0 × 10 /L is relatively unusual and offers a clue to the underlying diagnosis. In 2003, we established a specialist service to diagnose unexplained eosinophilia. Read More

Cancers (Basel) 2021 May 26;13(11). Epub 2021 May 26.

Department of Biomedical Science, Institute of Pharmacology and Toxicology, University of Veterinary Medicine Vienna, 1210 Vienna, Austria.

The Janus kinase-signal transducer and activator of transcription (JAK-STAT) pathway propagates signals from a variety of cytokines, contributing to cellular responses in health and disease. Gain of function mutations in JAKs or STATs are associated with malignancies, with being the main driver mutation in myeloproliferative neoplasms (MPN). Therefore, inhibition of this pathway is an attractive therapeutic strategy for different types of cancer. Read More

Diagnostics (Basel) 2021 May 27;11(6). Epub 2021 May 27.

Department of Bioengineering, University of California, San Diego, CA 92093-0412, USA.

The red blood cell has become implicated in the progression of a range of diseases; mechanisms by which red cells are involved appear to include the transport of inflammatory species via red cell-derived vesicles. We review this role of RBCs in diseases such as diabetes mellitus, sickle cell anemia, polycythemia vera, central retinal vein occlusion, Gaucher disease, atherosclerosis, and myeloproliferative neoplasms. We propose a possibly unifying, and novel, paradigm for the inducement of RBC vesiculation during vascular flow of red cells adhered to the vascular endothelium as well as to the red pulp of the spleen. Read More

Curr Oncol 2021 May 10;28(3):1790-1794. Epub 2021 May 10.

Department of Human and Animal Cell Lines, Leibniz-Institute DSMZ-German Collection of Microorganisms and Cell Cultures, 38124 Braunschweig, Germany.

Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative neoplasm that is genetically characterized by the absence of both the Philadelphia chromosome and BCR-ABL1 fusion gene and the high prevalence of mutations in the colony-stimulating factor 3 receptor (CSF3R). Additional disease-modifying mutations have been recognized in CNL samples, portraying a distinct mutational landscape. Despite the growing knowledge base on genomic aberrations, further progress could be gained from the availability of representative models of CNL. Read More

Cancers (Basel) 2021 May 22;13(11). Epub 2021 May 22.

Department of Medicine and Surgery, Anatomy Unit, University of Parma, 43126 Parma, Italy.

Single nucleotide polymorphisms (SNPs) can modify the individual pro-inflammatory background and may therefore have relevant implications in the MPN setting, typified by aberrant cytokine production. In a cohort of 773 primary myelofibrosis (PMF), we determined the contribution of the rs1024611 SNP of CCL2-one of the most potent immunomodulatory chemokines-to the clinical and biological characteristics of the disease, demonstrating that male subjects carrying the homozygous genotype G/G had an increased risk of PMF and that, among PMF patients, the G/G genotype is an independent prognostic factor for reduced overall survival. Functional characterization of the SNP and the CCL2-CCR2 axis in PMF showed that i) homozygous PMF cells are the highest chemokine producers as compared to the other genotypes; ii) PMF CD34+ cells are a selective target of CCL2, since they uniquely express CCR2 (CCL2 receptor); iii) activation of the CCL2-CCR2 axis boosts pro-survival signals induced by driver mutations via Akt phosphorylation; iv) ruxolitinib effectively counteracts CCL2 production and down-regulates CCR2 expression in PMF cells. Read More

Cancers (Basel) 2021 May 12;13(10). Epub 2021 May 12.

Department of Medicine, General Pathology Division, University of Verona, 37134 Verona, Italy.

Chronic myeloid leukemia (CML) is a myeloproliferative disorder characterized by BCR-ABL1 oncogene expression. This dysregulated protein-tyrosine kinase (PTK) is known as the principal driver of the disease and is targeted by tyrosine kinase inhibitors (TKIs). Extensive documentation has elucidated how the transformation of malignant cells is characterized by multiple genetic/epigenetic changes leading to the loss of tumor-suppressor genes function or proto-oncogenes expression. Read More

Int J Mol Sci 2021 May 9;22(9). Epub 2021 May 9.

Patrick G Johnston Center for Cancer Research, Queens University Belfast, 97 Lisburn Road, Belfast BT9 7AE, UK.

Myeloid malignancy is a broad term encapsulating myeloproliferative neoplasms (MPN), myelodysplastic syndrome (MDS) and acute myeloid leukaemia (AML). Initial studies into genomic profiles of these diseases have shown 2000 somatic mutations prevalent across the spectrum of myeloid blood disorders. Epigenetic mutations are emerging as critical components of disease progression, with mutations in genes controlling chromatin regulation and methylation/acetylation status. Read More

Am Fam Physician 2021 Jun;103(11):680-687

University of Tennessee College of Medicine, Chattanooga, TN, USA.

Polycythemia vera is one of three stem-cell-derived myeloid malignancies commonly known as myeloproliferative neoplasms. It is characterized by erythrocytosis, often with associated leukocytosis and thrombocytosis. It has a significant negative impact on overall mortality and morbidity in the form of arterial and venous clots, symptoms of fatigue and pruritus, and conversion to leukemia and myelofibrosis. Read More

Br J Haematol 2021 Jun 1. Epub 2021 Jun 1.

Haematology and Oncology, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany.

We report on 45 patients with myeloid neoplasms and concurrent Janus kinase 2 (JAK2) V617F and KIT proto-oncogene, receptor tyrosine kinase (KIT) D816V (JAK2 /KIT ) mutations, which are individually identified in >60% of patients with classical myeloproliferative neoplasms (MPN) and >90% of patients with systemic mastocytosis (SM) respectively. In SM, the concurrent presence of a clonal non-mast cell neoplasm [SM with associated haematological neoplasm (SM-AHN)] usually constitutes a distinct subtype associated with poor survival. All 45 patients presented with a heterogeneous combination of clinical/morphological features typical of the individual disorders (e. Read More

Br J Haematol 2021 Jun 1. Epub 2021 Jun 1.

Department of Hematology and Oncology, Matsusaka Chuo General Hospital, Matsusaka, Mie, Japan.

Semin Thromb Hemost 2021 May 31. Epub 2021 May 31.

Department of Clinical Biochemistry, Aarhus University Hospital, Aarhus, Denmark.

Bleeding and thrombosis are well-known complications to hematological malignancies, and changes in fibrinolysis impact both these issues. In the present systematic review, we provide an overview and discussion of the current literature in regards to clinical manifestations, diagnosis, and treatment of altered fibrinolysis in patients suffering from hematological malignancies, beyond acute promyelocytic leukemia. We performed a systematic literature search employing the databases Pubmed, Embase, and Web of Science to identify original studies investigating fibrinolysis in hematological malignancies. Read More

J Int Med Res 2021 May;49(5):3000605211018426

The First Hospital of Jilin University, Changchun, China.

Myelodysplastic/myeloproliferative neoplasms (MDS/MPNs) are a heterogeneous group of hematologic malignancies characterized by dysplastic and myeloproliferative overlapping features in the bone marrow and blood. The occurrence of the disease is related to age, prior history of MPN or MDS, and recent cytotoxic or growth factor therapy, but it rarely develops after acute myeloid leukemia (AML). We report a rare case of a patient diagnosed with AML with t(8; 21)(q22; q22) who received systematic chemotherapy. Read More

Case Rep Oncol 2021 Jan-Apr;14(1):690-694. Epub 2021 Apr 15.

Department of Hematology and Medical Oncology, Hamad Medical Corporation, Doha, Qatar.

Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm (MPN) that harbors the Philadelphia chromosomal translocation resulting in the uncontrolled production of mature granulocytes. Commonly, patients are diagnosed with CML during blood work for other reasons or enlarged spleen. The diagnosis is based on WHO criteria that require the demonstration of Philadelphia chromosome. Read More

Hematology 2021 Dec;26(1):408-414

State Key Laboratory of Fine Chemicals, School of Life Science and Technology, Dalian University of Technology, Dalian, People's Republic of China.

Background: Chronic myelogenous leukemia (CML) is one of the most common cancers in the world. Imatinib is one of the most effective therapeutic strategies to inhibit the BCR-ABL tyrosine Kinase in patients with CML, but resistance is increasingly encountered.

Material And Methods: Microarray data GSE7114, GSE92624 and GSE97562 were downloaded and analyzed from Gene Expression Omnibus (GEO) to identify the candidate genes in the imatinib-resistant CML cells. Read More

West Afr J Med 2021 May;38(5):502-506

Department of Internal Medicine, Faculty of Clinical Medicine, College of Health Sciences, Ebonyi State University, Abakaliki, Ebonyi State, Nigeria.

Deafness occurs rarely in patients with chronic myeloid leukaemia. Hydroxyurea-induced leg ulcer has been found in patients on long-term hydroxyurea therapy. We present a 53 year old man who developed spontaneous bilateral deafness shortly after he was diagnosed with chronic myeloid leukaemia and subsequently developed hydroxyurea induced leg ulcer in the course of treatment. Read More

Prog Mol Subcell Biol 2021 ;59:163-180

Departments of Immunobiology, Yale University School of Medicine, New Haven, CT, USA.

The endoplasmic reticulum (ER) performs key cellular functions including protein synthesis, lipid metabolism and signaling. While these functions are spatially isolated in structurally distinct regions of the ER, there is cross-talk between the pathways. One vital player that is involved in ER function is the ER-resident protein calreticulin (CALR). Read More

J Nucl Med 2021 May 28. Epub 2021 May 28.

Nuclear Medicine, Medical Faculty, University of Augsburg, Germany.

C-X-C motif chemokine receptor 4 (CXCR4) is an attractive target for cancer diagnosis and treatment, as it is overexpressed in many solid and hematological malignancies. This study investigated the feasibility of CXCR4-directed imaging with positron emission tomography/computed tomography (PET/CT) using Ga-Pentixafor to visualize and quantify disease involvement in myeloproliferative neoplasms (MPNs). 12 patients with MPNs ( = 4 primary myelofibrosis, = 6 essential thrombocythemia, = 2 polycythemia vera) and 5 controls underwent Ga-Pentixafor-PET/CT. Read More

Klin Lab Diagn 2021 May;66(5):315-320

Central Research Institute of Epidemiology Rospotrebnadzor.

Somatic mutations associated with oncological diseases, including Ph-myeloproliferative neoplasms (Ph-MPN), are very diverse, occur with different frequencies and different allelic burden levels. Therefore, at the initial stage of performing molecular-genetic diagnostic procedures, it is desirable to be able to conduct screening tests in the laboratory. This is especially important when analyzing rare and diverse mutations. Read More

Leuk Res Rep 2021 8;15:100243. Epub 2021 May 8.

Clinica Ematologica, Ospedali Riuniti di Ancona, Ancona, Italy.

Major disease complications for patients with essential thrombocythemia (ET) include thrombosis and fibrotic or leukemic transformation. Calreticulin (CALR) mutation type 1 frequencies in ET are estimated between 7% and 11% and ET patients carrying type 1 mutation are associated with lower risk of thrombosis but higher risk of myelofibrosis transformation compared to ET patients with mutation. Leukemic transformation rates at 20 years are estimated at less than 5% for ET and risk factors for leukemic transformation are advanced age, thrombosis history, leukocytosis, and anemia. Read More

J Clin Pathol 2021 May 26. Epub 2021 May 26.

Patrick G Johnston Centre for Cancer Research (PGJCCR), Queen's University Belfast, Belfast, UK.

The leucocyte common antigen, protein tyrosine phosphatase receptor type C (PTPRC), also known as CD45, is a transmembrane glycoprotein, expressed on almost all haematopoietic cells except for mature erythrocytes, and is an essential regulator of T and B cell antigen receptor-mediated activation. Disruption of the equilibrium between protein tyrosine kinase and phosphatase activity (from CD45 and others) can result in immunodeficiency, autoimmunity, or malignancy. CD45 is normally present on the cell surface, therefore it works upstream of a large signalling network which differs between cell types, and thus the effects of CD45 on these cells are also different. Read More