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Chronic myeloproliferative neoplasm

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https://www.readbyqxmd.com/read/27899359/how-i-treat-atypical-chronic-myeloid-leukemia
#1
Jason Gotlib
Atypical chronic myeloid leukemia, BCR-ABL1-negative (aCML) is a rare myelodysplastic syndrome/myeloproliferative neoplasm for which no current standard of care exists. The challenges of atypical CML relate to its heterogeneous clinical and genetic features, high rate of transformation to acute myeloid leukemia, and historically poor survival. Therefore, allogeneic hematopoietic stem cell transplantation should always be an initial consideration for eligible patients with a suitable donor. Non-transplant approaches for treating aCML have otherwise largely relied on adopting treatment strategies used for MDS and MPN...
November 29, 2016: Blood
https://www.readbyqxmd.com/read/27884974/from-leeches-to-personalized-medicine-evolving-concepts-in-the-management-of-polycythemia-vera
#2
Alessandro Maria Vannucchi
Polycythemia vera (PV), a clonal disorders of hematopoietic stem/progenitor cells that manifests with prevalent expansion of red cell mass, is the most frequent among chronic myeloproliferative neoplasms (MPN). It is characterized by a V617F point mutation in JAK2 exon 14 , or less common mutations in exon 12, in virtually all cases. The landmark discovery of autonomously activated JAK/STAT signaling pathway paved the way for the clinical development of the first target drug, the JAK1 and JAK2 inhibitor ruxolitinib, that is now approved for patients with resistance or intolerance to hydroxyurea...
November 24, 2016: Haematologica
https://www.readbyqxmd.com/read/27875375/management-of-venous-thromboembolism-in-myeloproliferative-neoplasms
#3
Tiziano Barbui, Valerio De Stefano
PURPOSE OF REVIEW: Venous thromboembolism is frequent in chronic myeloproliferative neoplasms (MPN). The current strategy for its management includes cytoreductive therapy and antithrombotic prophylaxis, but many issues remain uncertain. In this review, the risk factors and prevention of recurrences will be discussed. RECENT FINDINGS: Around one-third of patients with polycythemia vera and essential thrombocythemia experience a major thrombosis at diagnosis or during follow-up...
November 21, 2016: Current Opinion in Hematology
https://www.readbyqxmd.com/read/27852053/therapy-related-acute-myeloid-leukemia-following-treatment-of-lymphoid-malignancies
#4
Sarah Bertoli, Arthur Sterin, Suzanne Tavitian, Lucie Oberic, Loïc Ysebaert, Reda Bouabdallah, François Vergez, Audrey Sarry, Emilie Bérard, Françoise Huguet, Guy Laurent, Thomas Prébet, Norbert Vey, Christian Récher
Therapy-related acute myeloid leukemia (t-AML) is a heterogeneous entity most frequently related to breast cancer or lymphoproliferative diseases (LD). Population-based studies have reported an increased risk of t-AML after treatment of lymphomas. The aim of this study was to describe the characteristics and outcome of 80 consecutive cases of t-AML following treatment of LD. t-AML accounted for 2.3% of all AML cases, occurred 60 months after LD diagnosis, and were characterized by a high frequency of FAB M6 AML and poor-risk cytogenetic abnormalities...
November 10, 2016: Oncotarget
https://www.readbyqxmd.com/read/27822101/the-danish-national-chronic-myeloid-neoplasia-registry
#5
REVIEW
Marie Bak, Else Helene Ibfelt, Thomas Stauffer Larsen, Dorthe Rønnov-Jessen, Niels Pallisgaard, Ann Madelung, Lene Udby, Hans Carl Hasselbalch, Ole Weis Bjerrum, Christen Lykkegaard Andersen
AIM: The Danish National Chronic Myeloid Neoplasia Registry (DCMR) is a population-based clinical quality database, introduced to evaluate diagnosis and treatment of patients with chronic myeloid malignancies. The aim is to monitor the clinical quality at the national, regional, and hospital departmental levels and serve as a platform for research. STUDY POPULATION: The DCMR has nationwide coverage and contains information on patients diagnosed at hematology departments from January 2010 onward, including patients with essential thrombocythemia, polycythemia vera, myelofibrosis, unclassifiable myeloproliferative neoplasms, chronic myelomonocytic leukemia, and chronic myeloid leukemia...
2016: Clinical Epidemiology
https://www.readbyqxmd.com/read/27812500/an-etv6-abl1-fusion-in-a-patient-with-chronic-myeloproliferative-neoplasm-initial-response-to-imatinib-followed-by-rapid-transformation-into-all
#6
Purvi M Kakadia, Ralf Schmidmaier, Andreas Völkl, Irene Schneider, Natalia Huk, Stephanie Schneider, Gerda Panzner, Ulrike Neidel, Barbara Fritz, Karsten Spiekermann, Stefan K Bohlander
We report the case of a 26 year-old patient presenting with a persistent leukocytosis and CML-like marrow but no evidence of a BCR/ABL1 fusion. Molecular cytogenetics revealed that a portion of the ETV6 locus was inserted into the ABL1 locus. An ETV6/ABL1 fusion transcript could subsequently be confirmed. The patient was started on imatinib and went into complete cytomorphological remission. QRT-PCR measurements showed a 4 log reduction of the ETV6/ABL1 fusion. 15 months later, the disease transformed into ALL and the patient expired...
2016: Leukemia Research Reports
https://www.readbyqxmd.com/read/27807503/clinical-management-of-myelodysplastic-syndrome-myeloproliferative-neoplasm-overlap-syndromes
#7
Joseph A Clara, David A Sallman, Eric Padron
The myelodysplastic/myeloproliferative neoplasms (MDS/MPNs) are a unique group of hematologic malignancies characterized by concomitant myelodysplastic and myeloproliferative features. According to the 2008 WHO classification, the category includes atypical chronic myeloid leukemia (aCML), chronic myelomonocytic leukemia (CMML), juvenile myelomonocytic leukemia (JMML), MDS/MPN-unclassifiable (MDS/MPN-U), and the provisional entity refractory anemia with ring sideroblasts and thrombocytosis (RARS-T). Although diagnosis currently remains based on clinicopathologic features, the incorporation of next-generation platforms has allowed for the recent molecular characterization of these diseases which has revealed unique and complex mutational profiles that support their distinct biology and is anticipated to soon play an integral role in diagnosis, prognostication, and treatment...
September 2016: Cancer Biology & Medicine
https://www.readbyqxmd.com/read/27806853/cutaneous-myeloid-dendritic-cell-dyscrasia-a-cutaneous-clonal-monocytosis-associated-with-chronic-myeloproliferative-disorders-and-peripheral-blood-monocytosis
#8
Cynthia M Magro, Shabnam Momtahen, Shalini Verma, Ronnie M Abraham, Constantin Friedman, Gerard J Nuovo, Wayne Tam
Monocytes are critical components of the innate immune system and they can differentiate into dendritic cells (DCs). Cutaneous neoplasms of dendritic cell origin are uncommon and mostly represented by histiocytic lesions derived primarily from Langerhans cells. The myeloid DC (mDC) while recognized in the immunology literature does not have a well-defined neoplastic cutaneous counterpart. Eleven patients with a diagnosis of cutaneous mDC dyscrasia were evaluated. Routine hematoxylin and eosin stain were performed followed by selective phenotypic studies...
December 2016: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/27797679/long-term-outcome-and-analysis-of-dysfunction-of-transjugular-intrahepatic-portosystemic-shunt-placement-in-chronic-primary-budd-chiari-syndrome
#9
Georges Hayek, Maxime Ronot, Aurélie Plessier, Annie Sibert, Mohamed Abdel-Rehim, Magaly Zappa, Pierre-Emmanuel Rautou, Dominique Valla, Valérie Vilgrain
Purpose To evaluate the long-term safety, technical success, and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) in a series of patients with Budd-Chiari syndrome (BCS), and to determine the predictors of shunt dysfunction. Materials and Methods From 2004 to 2013, all patients with primary BCS referred for TIPS placement were included in the study. The primary and secondary technical success rates and the number and types of early (ie, before day 7) complications were noted. Factors associated with dysfunction were analyzed with uni- and multivariate analyses...
October 31, 2016: Radiology
https://www.readbyqxmd.com/read/27796499/underlying-mechanisms-of-the-jak2v617f-mutation-in-the-pathogenesis-of-myeloproliferative-neoplasms
#10
A Mullally
Chronic myeloproliferative neoplasms (MPN) comprise a spectrum of clonal neoplastic disorders characterized by overproduction of terminally differentiated cells of the myeloid lineage. A common genetic basis for the BCR-ABL-negative MPN disorders was elucidated in 2005 with the identification of the JAK2V617F mutation in the majority of MPN patients. The discovery of JAK2V617F had a dramatic impact on the diagnosis and treatment of MPN. Testing for JAK2 mutations is now included in the World Health Organization (WHO) criteria for the diagnosis of MPN, and in 2011 the oral JAK2 kinase inhibitor ruxolitinib became the first Food and Drug Administration (FDA)-approved drug for the treatment of myelofibrosis...
October 31, 2016: Der Pathologe
https://www.readbyqxmd.com/read/27777768/jak2-v617f-mutation-multiple-hematologic-and-non-hematologic-processes-an-association
#11
Kenneth G Liu, Amit Verma, Olga Derman, Noah Kornblum, Murali Janakiram, Ira Braunschweig, Ramakrishna Battini
BACKGROUND: Population studies showed that patients with JAK2 V617F mutation had increased mortality, and increased risk of any cancer, hematologic cancer, and myeloproliferative disease. CASE PRESENTATION: A 68-year-old Asian male with JAK2 V617F mutation developed four different hematologic and non-hematologic neoplastic processes. In 2009, he was diagnosed with stage IA lung adenocarcinoma and also noted to have worsening leukocytosis and thrombocytosis with peak platelet count of 1,054,000/mL)...
2016: Biomarker Research
https://www.readbyqxmd.com/read/27766065/thrombopoietin-and-its-receptor-in-normal-and-neoplastic-hematopoiesis
#12
Kenneth Kaushansky
Thrombopoietin was posited to exist in 1958 and cloned in 1994, and in the ensuing two decades we have learned a great deal about the physiology and pathology of the primary regulator of thrombopoiesis. This paper will review the role of the hormone and its receptor, the product of the c-Mpl proto-oncogene, in health and disease, including many unexpected effects in both normal and neoplastic hematopoiesis. Amongst these unexpected properties are a non-redundant effect on hematopoietic stem cells, a critical role in all three of the acquired, chronic myeloproliferative neoplasms, as well as both gain-of-function and loss-of-function mutations in congenital and acquired states of thrombocytopenia and thrombocythemia...
2016: Thrombosis Journal
https://www.readbyqxmd.com/read/27764253/differential-dynamics-of-calr-mutant-allele-burden-in-myeloproliferative-neoplasms-during-interferon-alfa-treatment
#13
Lasse Kjær, Sabrina Cordua, Morten O Holmström, Mads Thomassen, Torben A Kruse, Niels Pallisgaard, Thomas S Larsen, Karin de Stricker, Vibe Skov, Hans C Hasselbalch
Discovery of somatic mutations in the calreticulin gene (CALR) has identified a subgroup of Philadelphia-negative chronic myeloproliferative neoplasms (MPN) with separate haematological characteristics and prognosis. CALR mutations serve as novel markers both of diagnostic value and as targets for monitoring molecular responses during therapy. Interferon-α (IFN) selectively targets the malignant clone in a subset of MPN patients and can induce both haematological and molecular remissions in CALR mutated essential thrombocythemia (ET) patients...
2016: PloS One
https://www.readbyqxmd.com/read/27760943/-chronic-myelomonocytic-leukemia-with-myelofibrosis-resulting-in-sudden-massive-pleural-effusion-during-cytoreductive-therapy-with-hydroxycarbamide
#14
Yoshitaka Sunami, Akihiko Gotoh, Naoki Watanabe, Yoko Edahiro, Yasuharu Hamano, Hironori Harada, Norio Komatsu
Pleural effusion may occur as a rare complication associated with myeloid hematological malignancies. However, it occasionally occurs in patients with myelodysplastic/myeloproliferative neoplasms(MDS/MPN), especially in chronic myelomonocytic leukemia(CMML)with marked leukocytosis. Pleural effusion can also develop in hematological disorders with bone marrow fibrosis. Here, we report a case of CMML with bone marrow fibrosis, in which massive pleural effusion developed rapidly during cytoreductive therapy with hydroxycarbamide(HU)...
October 2016: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/27745871/preferences-of-patients-with-myeloproliferative-neoplasms-for-accepting-anxiety-or-depression-treatment
#15
Daniel C McFarland, Megan Johnson Shen, Heather Polizzi, John Mascarenhas, Marina Kremyanskaya, Jimmie Holland, Ronald Hoffman
BACKGROUND: Patients with chronic hematologic malignancies such as myeloproliferative neoplasms suffer from significant physical and psychological symptom burden. This study examined their willingness to accept an antidepressant and their preferences for which provider (mental health professional or hematologist/oncologist) prescribes an antidepressant for the management of anxiety and depression. METHODS: Anxiety and depression treatment preferences were measured with 3 questions assessing: (1) willingness to accept an antidepressant, (2) willingness to have their hematologist/oncologist prescribe the antidepressant, and (3) preference for treatment by a psychiatrist or mental health professional...
August 17, 2016: Psychosomatics
https://www.readbyqxmd.com/read/27742771/genomic-analysis-of-myeloproliferative-neoplasms-in-chronic-and-acute-phases
#16
Frédéric Courtier, Nadine Carbuccia, Séverine Garnier, Arnaud Guille, José Adelaïde, Nathalie Cervera, Veronique Gelsi-Boyer, Marie-Joelle Mozziconacci, Jérôme Rey, Nobert Vey, Max Chaffanet, Daniel Birnbaum, Anne Murati
No abstract text is available yet for this article.
October 14, 2016: Haematologica
https://www.readbyqxmd.com/read/27725592/myeloproliferative-neoplasms-updates-on-molecular-pathophysiology-diagnosis-and-treatment-strategies
#17
Katsuto Takenaka
Myeloproliferative neoplasms (MPNs) are chronic hematopoietic stem cell disorders, including polycythemia vera, essential thrombocytosis, and primary myelofibrosis. The JAK2V617F mutation was identified in 2005, followed by the discovery of the JAK2 exon12, MPNW515 mutation, and CALR mutation. About 90% of patients with BCR/ABL negative MPNs have been shown to have one of these driver mutations. In addition, mutations in epigenetic regulators and RNA splicing genes were found to co-exist with driver mutations and to play critical roles in the disease progression of MPNs...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27710990/coexistence-of-chronic-lymphocytic-leukemia-and-polycythemia-vera-a-case-report-and-review-of-the-literature
#18
Serdal Korkmaz, Sinan Kulakoglu, Hasan Gorkem, Bilal Aygun, Ali Cetinkaya
: Polycythemia vera is a Philadelphia chromosome-negative myeloproliferative neoplasm. Chronic lymphocytic leukemia is a monoclonal expansion of a CD5+ CD19+ B lymphocytes. Chronic myeloproliferative neoplasms may coexist with indolent B-cell malignant lymphomas of various types. The association of chronic lymphocytic leukemia with polycythemia vera is a rare event with only a few cases of coexistence ever reported. We report a 56-year-old man in whom these two disorders were diagnosed concomitantly...
September 2016: Annals of Saudi Medicine
https://www.readbyqxmd.com/read/27707735/when-clinical-heterogeneity-exceeds-genetic-heterogeneity-thinking-outside-the-genomic-box-in-chronic-myelomonocytic-leukemia
#19
Markus Ball, Alan F List, Eric Padron
Exome sequencing studies in Chronic Myelomonocytic Leukemia (CMML) illustrate a mutational landscape characterized by few somatic mutations involving a subset of recurrent gene mutations in ASXL1, SRSF2, and TET2, each approaching 40% in incidence. This has led to the clinical implementation of next generation sequencing panels that effectively identify clonal monocytosis and complement clinical prognostic scoring systems in most patients. However, most murine models based on single gene mutations fail to recapitulate the CMML phenotype and many gene mutations are loss-of-function making the identification of traditional therapeutic vulnerabilities challenging...
October 5, 2016: Blood
https://www.readbyqxmd.com/read/27687869/allogeneic-hematopoietic-stem-cell-transplant-in-adult-patients-with-myelodysplastic-syndrome-myeloproliferative-neoplasm-mds-mpn-overlap-syndromes
#20
Prashant Sharma, Shivani S Shinde, Moussab Damlaj, Mehrdad Hefazi Rorghabeh, Shahrukh K Hashmi, Mark R Litzow, William J Hogan, Naseema Gangat, Michelle A Elliott, Aref Al-Kali, Ayalew Tefferi, Mrinal M Patnaik
MDS/MPN (myelodysplastic syndrome/myeloproliferative neoplasm) overlap syndromes are myeloid malignancies for which allogeneic hematopoietic stem cell transplant (allo-HSCT) is potentially curative. We describe transplant outcomes of 43 patients - 35 with chronic myelomonocytic leukemia, CMML (of which 17 had blast transformation, BT) and eight with MDS/MPN-unclassifiable (MDS/MPN,U). At median follow-up of 21 months, overall survival (OS), cumulative incidence of relapse (CIR) and non-relapse mortality (NRM) were 55%, 29%, and 25% respectively in CMML without BT and 47%, 40%, and 34% respectively in CMML with BT...
August 11, 2016: Leukemia & Lymphoma
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