keyword
MENU ▼
Read by QxMD icon Read
search

Myeloproliferative disorder

keyword
https://www.readbyqxmd.com/read/29225884/setbp1-mutations-as-a-biomarker-for-myelodysplasia-myeloproliferative-neoplasm-overlap-syndrome
#1
REVIEW
Katherine Linder, Chaitanya Iragavarapu, Delong Liu
Myelodysplasia (MDS) /myeloproliferative neoplasm (MPN) overlap syndrome has been described since the 2001 WHO classification as disorders that have both proliferative and dysplastic changes simultaneously. Specific disorders include chronic myelomonocytic leukemia (CMML), juvenile myelomonocytic leukemia (JMML), BCR-ABL negative atypical chronic myeloid leukemia (aCML) and unclassifiable MDS/MPN (MPN/MDS-U). Recurrent gene mutations in these conditions have been described. Among them, SETBP1 mutations have been identified in up to 32% of aCML, 24% of JMML, 18% of CMML and 10% of MDS/MPN-U patients...
2017: Biomarker Research
https://www.readbyqxmd.com/read/29223988/prevalence-of-physical-problems-detected-by-the-distress-thermometer-and-problem-list-in-patients-with-myeloproliferative-disorders
#2
Daniel C McFarland, Kelly M Shaffer, Heather Polizzi, John Mascarenhas, Marina Kremyanskaya, Jimmie Holland, Ronald Hoffman
Background: Patients with myeloproliferative neoplasms (MPNs) can have a severe physical symptom burden over an extended disease trajectory that contributes to decreased quality of life. Few studies, however, have characterized which patients most frequently consider physical symptoms a problem. This study describes the physical symptoms of patients with MPNs and the relationship of these symptoms to patient characteristics. Methods: Patients with MPNs (N=117) completed questionnaires in a dedicated academic medical center MPN clinic...
December 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/29222296/what-are-the-current-treatment-approaches-for-patients-with-polycythemia-vera-and-essential-thrombocythemia
#3
REVIEW
Alessandro M Vannucchi, Paola Guglielmelli
Polycythemia vera (PV) and essential thrombocythemia (ET) are chronic myeloproliferative neoplasms that are characterized by thrombohemorrhagic complications, symptom burden, and impaired survival mainly due to thrombosis, progression to myelofibrosis, and transformation to acute leukemia. In this manuscript, we will review the most recent changes in diagnostic criteria, the improvements in risk stratification, and the "state of the art" in the daily management of these disorders. The role of conventional therapies and novel agents, interferon α and the JAK2 inhibitor ruxolitinib, is critically discussed based on the results of a few basic randomized clinical studies...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222208/hypersomatotropism-induced-secondary-polycythaemia-leading-to-spontaneous-pituitary-apoplexy-resulting-in-cure-of-acromegaly-and-remission-of-polycythaemia-the-virtuous-circle
#4
Shinjan Patra, Sugata Narayan Biswas, Joydip Datta, Partha Pratim Chakraborty
A young man with subtle clinical features suggestive of hypersomatotropism presented with acute-onset severe headache. Relevant investigations confirmed polycythaemia and growth hormone (GH)-secreting pituitary macroadenoma with apoplexy. Secondary polycythaemia and myeloproliferative disorders were ruled out. At follow-up after 3 months, resolution of polycythaemia and acromegaly was observed, evident on normal haemoglobin levels, a normocellular marrow, and normal insulin-like growth factor-1 (IGF-1) with glucose-suppressed GH levels...
December 7, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29218307/calreticulin-challenges-posed-by-the-intrinsically-disordered-nature-of-calreticulin-to-the-study-of-its-function
#5
Lilian Varricchio, Mario Falchi, Massimiliano Dall'Ora, Caterina De Benedittis, Alessandra Ruggeri, Vladimir N Uversky, Anna Rita Migliaccio
Calreticulin is a Ca2+-binding chaperone protein, which resides mainly in the endoplasmic reticulum but also found in other cellular compartments including the plasma membrane. In addition to Ca2+, calreticulin binds and regulates almost all proteins and most of the mRNAs deciding their intracellular fate. The potential functions of calreticulin are so numerous that identification of all of them is becoming a nightmare. Still the recent discovery that patients affected by the Philadelphia-negative myeloproliferative disorders essential thrombocytemia or primary myelofibrosis not harboring JAK2 mutations carry instead calreticulin mutations disrupting its C-terminal domain has highlighted the clinical need to gain a deeper understanding of the biological activity of this protein...
2017: Frontiers in Cell and Developmental Biology
https://www.readbyqxmd.com/read/29201484/intracranial-myeloid-sarcoma-metastasis-mimicking-acute-subdural-hematoma
#6
Amandip S Gill, Rabina Gill, Paul Kaloostian, Dina Elias, John S Roufail, Aurora S Cruz, Panayiotis E Pelargos, Frank P K Hsu, Ronald C Kim, Robert E Ayer, Samer Ghostine
Myeloid sarcoma, a rare consequence of myeloproliferative disorders, is rarely seen in the central nervous system, most commonly in the pediatric population. Although there are a handful of case reports detailing initial presentation of CNS myeloid sarcoma in the adult population, we have been unable to find any reports of CNS myeloid sarcoma presenting as a large mass lesion in a herniating patient. Here, we present the case of a patient transferred to our facility for a very large subdural hematoma. Based on imaging characteristics, it was felt to be a spontaneous hematoma secondary to coagulopathy...
2017: Case Reports in Surgery
https://www.readbyqxmd.com/read/29198847/first-delivery-in-a-leukemia-survivor-after-transplantation-of-cryopreserved-ovarian-tissue-evaluated-for-leukemia-cells-contamination
#7
Moran Shapira, Hila Raanani, Iris Barshack, Ninette Amariglio, Sanaz Derech-Haim, Meital Nagar Marciano, Eyal Schiff, Raoul Orvieto, Dror Meirow
OBJECTIVE: To describe a successful autologous ovarian tissue re-transplantation in a sterile leukemia survivor after evaluation for minimal residual disease and provide a review of the current literature. DESIGN: Presentation of a carefully designed workup taken to evaluate tissue for minimal residual disease, its limitations, and applicability to other patients. To date, there have not been any publications of auto-transplantations in leukemia survivors, owing to an estimated high risk for malignancy induction...
November 29, 2017: Fertility and Sterility
https://www.readbyqxmd.com/read/29183180/a-spotlight-on-the-management-of-complications-associated-with-myeloproliferative-neoplasms-a-clinician-s-perspective
#8
Graeme Greenfield, Mary F McMullin
Myeloproliferative neoplasms (MPNs) are associated with a variety of symptoms and signs which cause major morbidity for the patients. The disorders are associated with increased incidence of thromboembolic and hemorrhagic events which can lead to complications and shortened life expectancy. Areas covered: Using systematic literature review and expert clinical and research experience the authors discuss strategies for the management of symptoms and signs including pruritus, fatigue, splenomegaly and cytopenia...
November 29, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/29145678/-thrombocytosis-and-thrombocytopenia-background-and-clinical-relevance
#9
Kai Wille, Parvis Sadjadian, Martin Griesshammer
Due to the central role of platelets in hemostasis, the clinical relevance of quantitative changes in platelet counts (< 150 G/l or > 450 G/l) may be significant. Thrombopoesis (= production of platelets) occurs in the bone marrow, and the hormone thrombopoetin takes control on its regulation.In thrombocytosis, primary causes have to be distinguished from the far more common reactive (= secondary) reasons. The most important form of primary thrombocytosis occurs in myeloproliferative neoplasms especially in essential thrombocythemia (ET)...
November 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29129488/dna-hypomethylating-agents-as-epigenetic-therapy-before-and-after-allogeneic-hematopoietic-stem-cell-transplantation-in-myelodysplastic-syndromes-and-juvenile-myelomonocytic-leukemia
#10
REVIEW
Christian Flotho, Sebastian Sommer, Michael Lübbert
Myelodysplastic syndrome (MDS) is a clonal bone marrow disorder, typically of older adults, which is characterized by ineffective hematopoiesis, peripheral blood cytopenias and risk of progression to acute myeloid leukemia. Juvenile myelomonocytic leukemia (JMML) is an aggressive myeloproliferative neoplasm occurring in young children. The common denominator of these malignant myeloid disorders is the limited benefit of conventional chemotherapy and a particular responsiveness to epigenetic therapy with the DNA-hypomethylating agents 5-azacytidine (azacitidine) or decitabine...
November 9, 2017: Seminars in Cancer Biology
https://www.readbyqxmd.com/read/29128551/transplant-decisions-in-patients-with-myelofibrosis-should-mutations-be-the-judge
#11
REVIEW
Rachel B Salit, H Joachim Deeg
The prognosis of myeloproliferative neoplasms (MPN), including primary myelofibrosis (PMF), polycythemia vera (PV; post-PV MF) and essential thrombocythemia (ET; post-EMF) varies considerably, between these disorders as well as within each diagnosis. Molecular studies have identified "driver mutations", in JAK2, MPL1 and CALR, and additional somatic DNA mutations, including ASXL1, EZH2, IDH1/2 and SRSF2, that affect prognosis differentially. Patients with mutations in CALR (type1) have a better outlook than patients with mutations in JAK2 or MPL, while patients without any of the driver mutations (triple negative) have the shortest life expectancy...
November 8, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29095762/acquired-hemophilia-with-thrombosis-in-a-cancer-patient-an-unusual-presentation
#12
Senem Maral, Sule Mine Bakanay, Imdat Dilek
: Acquired hemophilia A (AHA) which presents with spontaneous severe intramuscular, mucosal and/or subcutaneous bleeding is a rare bleeding disorder. Even 50% of AHA patients are defined as idiopathic; 10% of cases are related with malignancy. Here, we present a case of AHA in a 43-year-old lady who was diagnosed with malignancy and venous thromboembolism on vena cava 2 years ago. To the best of our knowledge, this is the first report in literature presented with both acquired hemophilia and thrombosis associated with malignancy...
November 1, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29079125/hereditary-persistence-of-hemoglobin-f-is-protective-against-red-cell-sickling-a-case-report-and-brief-review
#13
Alexandra Sokolova, Anton Mararenko, Alexander Rozin, Alida Podrumar, Vladimir Gotlieb
Fetal hemoglobin (HbF) is a physiologic protein tetramer that is crucial for a developing fetus to survive in utero. Maternal hemoglobin has a relatively lower affinity for oxygen, and thus allows for an efficient transfer of oxygen from maternal to fetal blood. In addition to fulfilling a critical physiologic role, HbF is also known to alleviate symptoms of sickle-cell disease (SCD). The concentration of HbF depends on several factors. HbF is elevated in inherited conditions, such as hereditary persistence of HbF, hereditary spherocytosis, and thalassemia...
October 16, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/29078922/myeloproliferative-disorders
#14
REVIEW
Brian Meier, John H Burton
The emergency providers generally encounters myeloproliferative disorders (MPNs) in 1 of 2 ways: as striking laboratory abnormalities of seeming unknown consequence, or in previously diagnosed patients presenting with complications. The course of patients with MPNs is highly variable, but major complications can arise. Emergent conditions related to hyperviscosity need to be recognized early and treated aggressively. Rapid hydration, transfusion, cytoreduction, and early hematology consultation can be lifesaving...
December 2017: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29076133/effects-of-vascular-endothelial-growth-factors-and-their-receptors-on-megakaryocytes-and-platelets-and-related-diseases
#15
REVIEW
Jin-Gang Yang, Li-Li Wang, Dong-Chu Ma
It is well known that vascular endothelial growth factors (VEGFs) and their receptors (vascular endothelial growth factor receptors, VEGFRs) are expressed in different tissues, and VEGF-VEGFR loops regulate a wide range of responses, including metabolic homeostasis, cell proliferation, migration and tubuleogenesis. As ligands, VEGFs act on three structurally related VEGFRs (VEGFR1, VEGFR2 and VEGFR3 [also termed FLT1, KDR and FLT4, respectively]) that deliver downstream signals. Haematopoietic stem cells (HSCs), megakaryocytic cell lines, cultured megakaryocytes (MKs), primary MKs and abnormal MKs express and secrete VEGFs...
October 26, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29046979/medical-management-of-pulmonary-hypertension-with-unclear-and-or-multifactorial-mechanisms-group-5-is-there-a-role-for-pulmonary-arterial-hypertension-medications
#16
REVIEW
Jason Weatherald, Laurent Savale, Marc Humbert
PURPOSE OF REVIEW: The purpose of this review was to outline the mechanisms and to review recent literature on pulmonary arterial hypertension (PAH) medications in group 5 pulmonary hypertension (PH). RECENT FINDINGS: The first steps in management are to understand the mechanisms and hemodynamic profile and to exclude chronic thromboembolic disease. Recent studies in the past 5 years have found that PAH medications may improve hemodynamics in patients with pre-capillary pulmonary hypertension due to sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, and myeloproliferative disorders with dasatinib-induced PH...
October 18, 2017: Current Hypertension Reports
https://www.readbyqxmd.com/read/29040257/current-challenges-in-the-management-of-essential-thrombocythemia
#17
Ariel Kleman, Arun K Singavi, Laura C Michaelis
Essential thrombocythemia (ET), an uncommon blood cancer, is one of the classic myeloproliferative neoplasms, a category that also includes polycythemia vera and primary myelofibrosis. All 3 diseases are clonal hematopoietic stem cell disorders. Since 2005, when scientists discovered a molecular aberration driving clonal hematopoiesis in polycythemia vera, our understanding of the genomic underpinnings of these conditions has increased rapidly. Over the last decades, primary prevention of thrombotic and hemorrhagic complications has improved the lives of patients with ET, and the ability to characterize the disease by the presence or absence of molecular mutations has lent precision to our prognostic models...
October 2017: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/29037480/linking-energy-sensing-to-suppression-of-jak-stat-signalling-a-potential-route-for-repurposing-ampk-activators
#18
REVIEW
Claire Speirs, Jamie J L Williams, Kirsten Riches, Ian P Salt, Timothy M Palmer
Exaggerated Janus kinase-signal transducer and activator of transcription (JAK-STAT) signalling is key to the pathogenesis of pro-inflammatory disorders, such as rheumatoid arthritis and cardiovascular diseases. Mutational activation of JAKs is also responsible for several haematological malignancies, including myeloproliferative neoplasms and acute lymphoblastic leukaemia. Accumulating evidence links adenosine 5'-monophosphate (AMP)-activated protein kinase (AMPK), an energy sensor and regulator of organismal and cellular metabolism, with the suppression of immune and inflammatory processes...
October 13, 2017: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/29036785/wide-transcriptome-analysis-and-cellularity-of-bone-marrow-cd34-lin-cells-of-patients-with-chronic-phase-chronic-myeloid-leukemia-at-diagnosis-vs-12-months-of-first-line-nilotinib-treatment
#19
Alessandra Trojani, Ester Pungolino, Giuseppe Rossi, Mariella D'Adda, Milena Lodola, Barbara Di Camillo, Alessandra Perego, Mauro Turrini, Ester Orlandi, Lorenza Borin, Alessandra Iurlo, Simona Malato, Francesco Spina, Maria Luisa Latargia, Francesco Lanza, Salvatore Artale, Michela Anghilieri, Maria Cristina Carraro, Gabriella De Canal, Enrica Morra, Roberto Cairoli
BACKGROUND: Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder with heterogeneous biological and clinical features. The biomolecular mechanisms of CML response to tyrosine-kinase inhibitors are not fully defined. OBJECTIVE: We undertook a gene expression profiling (GEP) study of selected bone marrow (BM) CD34+/lin- cells of chronic-phase CML patients at diagnosis and after 12 months of TKI nilotinib to investigate molecular signatures characterizing both conditions...
September 29, 2017: Cancer Biomarkers: Section A of Disease Markers
https://www.readbyqxmd.com/read/28991281/splanchnic-vein-thrombosis-as-a-first-manifestation-of-primary-myelofibrosis
#20
Gregorio Campos-Cabrera, Virginia Campos-Cabrera, Salvador Campos-Cabrera, José-Luis Campos-Villagómez, Alejandra Romero-González
Myeloproliferative neoplasms are chronic disorders of clonal hematopoietic stem cells, characterized by an overproduction of functional granulocytes, red blood cells and / or platelets, and one of the major complications is the occurrence of venous and arterial thrombotic problems caused by increased platelet aggregation and thrombin generation. In this study 11 cases of primary myelofibrosis (PM) were evaluated and 2 debuted with splanchnic venous thrombosis (SVT); so after seeing the results of this study and of world literature, it is suggested that in patients with SVT, diagnostic methods for PM like the JAK2V617F mutation should be included...
2017: Gaceta Médica de México
keyword
keyword
6599
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"