50,098 results match your criteria myeloproliferative disorder


Cancer-Associated Splanchnic Vein Thrombosis.

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

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[Chronic-phase chronic myeloid leukemia with intracranial hemorrhage complicated with tumor lysis syndrome].

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

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Development of a method for the determination of the JAK2 gene mRNA in venous blood and assessment of its diagnostic value in oncohematology.

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

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New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome.

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

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Current Views on the Interplay between Tyrosine Kinases and Phosphatases in Chronic Myeloid Leukemia.

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

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The Impact of Epigenetic Modifications in Myeloid Malignancies.

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

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Clinical and histopathological features of myeloid neoplasms with concurrent Janus kinase 2 (JAK2) V617F and KIT proto-oncogene, receptor tyrosine kinase (KIT) D816V mutations.

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

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Altered Fibrinolysis in Hematological Malignances.

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

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Screening and identification of key genes in imatinib-resistant chronic myelogenous leukemia cells: a bioinformatics study.

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

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December 2021

Chronic Myeloid Leukaemia with Sudden Bilateral Deafness and Leg Ulcer Associated with Hydroxyurea Therapy.

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

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Screening of somatic mutations in the JAK2 and CALR genes by high-resolution melting curve analysis.

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

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Protein tyrosine phosphatase receptor type C (PTPRC or CD45).

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

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Tanshinone I inhibited growth of human chronic myeloid leukemia cells via JNK/ERK mediated apoptotic pathways.

Braz J Med Biol Res 2021 24;54(8):e10685. Epub 2021 May 24.

The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, China.

Tanshinone I (Tan I) is one of the main bioactive ingredients derived from Salvia miltiorrhiza Bunge, which has exhibited antitumor activities toward various human cancer cells. However, its effects and underlying mechanisms on human chronic myeloid leukemia (CML) cells still require further investigation. This study determined the effects and mechanisms of anti-proliferative and apoptosis induction activity induced by Tan I against K562 cells. Read More

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Eye: the door to undiagnosed chronic myeloid leukaemia.

BMJ Case Rep 2021 May 24;14(5). Epub 2021 May 24.

Vitreoretinal Services, LV Prasad Eye Institute Bhubaneswar Campus, Bhubaneswar, Odisha, India.

Chronic myeloid leukaemia (CML) is a myeloproliferative neoplasm that can present in varied ways from incidental finding on haemogram to symptomatic presentation such as splenomegaly. We report an interesting case of a 22-year-old man who presented with loss of vision in right eye for 1 month. There were no pre-existing ocular or systemic diseases. Read More

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A unique three-way Philadelphia chromosome variant t(4;9;22)(q21;q34;q11.2) in a newly diagnosed patient with chronic phase chronic myeloid leukemia: a case report and review of the literature.

J Med Case Rep 2021 May 25;15(1):285. Epub 2021 May 25.

Department of Internal Medicine III, Division of Hematology and Cell Therapy, Yamagata University faculty of Medicine, 2-2-2 Iida-Nishi, Yamagata, 990-9585, Japan.

Background: Chronic myeloid leukemia is a hematologic malignancy associated with the fusion of two genes: BCR and ABL1. This fusion results from a translocation between chromosomes 9 and 22, which is called the Philadelphia chromosome. Although the Philadelphia chromosome is present in more than 90% of patients with chronic myeloid leukemia, 5-8% of patients with chronic myeloid leukemia show complex variant translocations. Read More

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Immunoglobulin G4-Related Disease Accompanied by Primary Myelofibrosis: Case Report.

Front Med (Lausanne) 2021 4;8:638794. Epub 2021 May 4.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Science, Beijing, China.

Immunoglobulin G4-related disease (IgG4-RD) is a heterogeneous autoimmune fibrosing disorder that presents common pathologic features but with unclear etiology. We report a rare case of IgG4-RD accompanied by primary myelofibrosis that eventually transformed into acute myeloid leukemia. A 50-year-old woman suffered from progressive lacrimal and parotid gland enlargement, diaphoresis, and rapid weight loss. Read More

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Successful thrombolysis in essential thrombocythemia-related acute ischaemic stroke.

BMJ Case Rep 2021 May 19;14(5). Epub 2021 May 19.

Neurology, All India Institute of Medical Sciences - Rishikesh, Rishikesh, Uttarakhand, India

Essential thrombocythemia (ET)-related acute ischaemic stroke (AIS) may account for approximately 0.25%-0.5% of all ischaemic strokes. Read More

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Low‒Risk Essential Thrombocythemia Who Presented with Recurrent Episodes of Cerebral Hemorrhage during Pregnancy and Developed Cerebral Infarction during Puerperium.

Gan To Kagaku Ryoho 2021 May;48(5):725-730

Dept. of Hematology, Juntendo University Urayasu Hospital.

A 42‒year‒old woman. At week 27 of pregnancy, she developed subcortical hemorrhage and underwent open cranial surgery for hematoma evacuation. The platelet(Plt)count was 297,000/μL. Read More

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Fifth-week immunogenicity and safety of anti-SARS-CoV-2 BNT162b2 vaccine in patients with multiple myeloma and myeloproliferative malignancies on active treatment: preliminary data from a single institution.

J Hematol Oncol 2021 05 17;14(1):81. Epub 2021 May 17.

Hematology Unit, Department of Research and Clinical Oncology, IRCCS Regina Elena National Cancer Institute, Via Elio Chianesi 53, 00144, Rome, Italy.

Background: Safety and immunogenicity of BNT162b2 mRNA vaccine are unknown in hematological patients; both were evaluated prospectively in 42 patients with multiple myeloma (MM) and 50 with myeloproliferative malignancies (MPM) (20 chronic myeloid leukemias and 30 myeloproliferative neoplasms), all of them on active anti-cancer treatment, in comparison with 36 elderly controls not suffering from cancer. Subjects serologically and/or molecularly (by nasal/throat swab) positives at basal for SARS-CoV-2 were excluded. Primary endpoint was to compare titers of neutralizing anti-SARS-CoV-2 IgG and seroprotection rates among the cohorts at 3 and 5 weeks from first dose. Read More

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Intracellular ROS profile in hematopoietic progenitors of MDS patients: association with blast count and iron overload.

Hematology 2021 Dec;26(1):88-95

Biological Sciences, Sunnybrook Research Institute, Sunnybrook Health Sciences Centre, Toronto, Canada.

Reactive oxygen species (ROS) are under scrutiny as a participant in the pathophysiology of myelodysplastic syndrome (MDS) and the progression of MDS to acute myeloid leukemia (AML). Measurement of intracellular ROS (iROS) is particularly important since iROS is a direct indicator of cellular health and integrity. We developed a technique to measure standardize iROS (siROS) level in lymphocytes and bone marrow (BM) CD34 hematopoietic progenitors using the fluorescent probe dichlorofluorescein (DCF). Read More

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December 2021

A clinical appraisal of chronic myeloid leukaemia (CML)-related death and CML-specific death-Are they synonymous?

Int J Clin Pract 2021 Jun;75(6):e14217

Division of Hematology, Department of Internal Medicine, Cerrahpaşa Faculty of Medicine, Istanbul University-Cerrahpaşa, Istanbul, Turkey.

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Clonal evolution and clinical implications of genetic abnormalities in blastic transformation of chronic myeloid leukaemia.

Nat Commun 2021 05 14;12(1):2833. Epub 2021 May 14.

Laboratory of DNA Information Analysis, Human Genome Center, Institute of Medical Science, The University of Tokyo, Tokyo, Japan.

Blast crisis (BC) predicts dismal outcomes in patients with chronic myeloid leukaemia (CML). Although additional genetic alterations play a central role in BC, the landscape and prognostic impact of these alterations remain elusive. Here, we comprehensively investigate genetic abnormalities in 136 BC and 148 chronic phase (CP) samples obtained from 216 CML patients using exome and targeted sequencing. Read More

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The Age-Old Conundrum With Thiopurines: Are the Accumulating Risks Greater Than Benefits?

Authors:
Christina Ha

Am J Gastroenterol 2021 Apr;116(4):671-672

1The F Widjaja Foundation Inflammatory Bowel Immunology Research Institute at Cedars-Sinai, Los Angeles, California, USA.

This editorial comments on the study by Khan et al. that describes potential risk of acute myeloid leukemia or myeloproliferative disorder among thiopurine therapy. Read More

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Sirtuin1 and Chronic Myeloid Leukemia: a Comprehensive Glance at Drug Resistance.

Clin Lab 2021 May;67(5)

Background: Chronic myeloid leukemia (CML) is a myeloproliferative disorder, which is caused by BCR-ABL fusion that has tyrosine kinase activity. The emergence of the first generation of tyrosine kinase inhibitors increased survival in patients. CML patients remain in silent phase for a long time by using drugs such as imatinib. Read More

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[Precision diagnostics and therapy in hematological malignancies].

Lakartidningen 2021 05 10;118. Epub 2021 May 10.

professor, överläkare, Karolinska institutet och Karolinska universitetssjukhuset.

Precision diagnostics and therapy have been implemented rather early in clinical hematology due to the easy accessibility of blood and bone marrow, allowing not only for consecutive genetic analysis at diagnosis, remission and relapse, but also for culturing these cells and testing new drugs in vitro. One contributing factor has also been the relatively low number of »driver« mutations in hematologic malignancies and that some of them are gain of function mutations that are relatively easy to target by drugs. Examples of this development are ABL1-, JAK2-, and FLT3-inhibitors for the treatment of chronic myeloid leukemia, myeloproliferative neoplasms, and acute myeloid leukemia, respectively. Read More

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[Predisposition and progression of myelodysplastic syndromes].

Authors:
Hideki Makishima

Rinsho Ketsueki 2021 ;62(4):278-288

Pathology and Tumor Biology, Graduate School of Medicine, Kyoto University.

Recent advances in sequencing technologies have increased the detection rate for identifying germline mutations that predispose an individual to various myeloid neoplasms and somatic mutations acquired during progression from myelodysplastic syndromes (MDS) to acute myeloid leukemia (AML). In addition to pediatric subjects, adult patients were analyzed in order to obtain a complete spectrum of driver mutations in germline cells and/or somatic tumor samples. As shown in several recent studies, such driver mutations are acquired in a gene-specific fashion. Read More

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Mastocytosis-derived extracellular vesicles deliver miR-23a and miR-30a into pre-osteoblasts and prevent osteoblastogenesis and bone formation.

Nat Commun 2021 05 5;12(1):2527. Epub 2021 May 5.

Mast Cell Biology Section, Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health (NIH), Bethesda, MD, USA.

Osteoporosis and other manifestations of bone disease are frequent in patients with systemic mastocytosis (SM) in association with the presence of mast cell infiltrates in bone marrow, although the mechanisms behind bone disease remain poorly understood. We find that extracellular vesicles (EVs) released by neoplastic mast cells and present in the serum of patients with SM (SM-EVs) block osteoblast differentiation and mineralization in culture, and when injected into mice diminish the expression of osteoblast markers, and trabecular bone volume and microarchitecture. We demonstrate that miRNA-30a and miRNA-23a, increased in SM-EVs and neoplastic mast cell-derived EVs, attenuate osteoblast maturation by suppressing expression of RUNX2 and SMAD1/5, essential drivers of osteogenesis. Read More

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Additional cytogenetic abnormalities in chronic myeloid leukaemia; an experience from Pakistan.

J Pak Med Assoc 2021 Feb;71(2(B)):633-635

Department of Haematology, National Institute of Blood Diseases & BMT, Karachi.

Objective: To evaluate the presence and characteristics of additional karyotype abnormalities in chronic myeloid leukaemia cases.

Methods: The cross-sectional study was conducted at the Department of Cytogenetics and Molecular Pathology, National Institute of Blood Diseases and Bone Marrow Transplant, Karachi, from May 2010 to September 2016 and comprised diagnosed chronic myeloid leukaemia patients regardless of age and gender. Baseline cytogenetic evaluation was done on overnight, 24-hrs un-stimulated and 72-hrs stimulated bone marrow cultures, and karyotypes were defined according to the International System for Human Cytogenetic Nomenclature2013. Read More

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February 2021

Myelodysplastic syndromes and overlap syndromes.

Authors:
Yoon Hwan Chang

Blood Res 2021 Apr;56(S1):S51-S64

Department of Laboratory Medicine, Seoul National University Hospital, Seoul, Korea.

Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal hematological neoplasms characterized by ineffective hematopoiesis, morphologic dysplasia, and cytopenia. MDS overlap syndromes include various disorders, such as myelodysplastic/myeloproliferative neoplasms and hypoplastic MDS with aplastic anemia characteristics. MDS overlap syndromes share the characteristics of other diseases, which make differential diagnoses challenging. Read More

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The clinical role of interferon alpha in Philadelphia-negative myeloproliferative neoplasms.

Blood Res 2021 Apr;56(S1):S44-S50

Division of Hematology & Medical Oncology, Department of Internal Medicine, Soonchunhyang University Seoul Hospital, Seoul, Korea.

Myeloproliferative neoplasms (MPNs) are clonal hematopoietic stem cell malignancies. Chronic inflammation and a dysregulated immune system are central to the pathogenesis and progression of MPNs. Interferon alpha (IFNα) was first used for the treatment of MPNs approximately 40 years ago. Read More

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