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https://www.readbyqxmd.com/read/27870387/primary-myelofibrosis-2017-update-on-diagnosis-risk-stratification-and-management
#1
Ayalew Tefferi
: Disease overview: Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) characterized by stem cell-derived clonal myeloproliferation that is often but not always accompanied by JAK2, CALR or MPL mutation, abnormal cytokine expression, bone marrow fibrosis, anemia, splenomegaly, extramedullary hematopoiesis (EMH), constitutional symptoms, cachexia, leukemic progression and shortened survival. DIAGNOSIS: Diagnosis is based on bone marrow morphology...
December 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27773929/a-randomized-study-of-pomalidomide-vs-placebo-in-persons-with-myeloproliferative-neoplasm-associated-myelofibrosis-and-rbc-transfusion-dependence
#2
A Tefferi, H K Al-Ali, G Barosi, T Devos, H Gisslinger, Q Jiang, J-J Kiladjian, R Mesa, F Passamonti, V Ribrag, G Schiller, A M Vannucchi, D Zhou, D Reiser, J Zhong, R P Gale
: RBC-transfusion-dependence is common in persons with myeloproliferative neoplasm (MPN)-associated myelofibrosis. Determine rates of RBC-transfusion-independence after therapy with pomalidomide vs. placebo in persons with MPN-associated myelofibrosis and RBC-transfusion-dependence. 252 subjects (intent-to-treat population) including 229 subjects confirmed by central review (modified intent-to-treat population) were randomly-assigned (2:1) to pomalidomide or placebo. Trialists and subjects were blinded to treatment allocation...
October 24, 2016: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/27768091/mpl-mutations-and-palpable-splenomegaly-are-independent-risk-factors-for-fibrotic-progression-in-essential-thrombocythemia
#3
M Haider, Y C Elala, N Gangat, C A Hanson, A Tefferi
No abstract text is available yet for this article.
October 21, 2016: Blood Cancer Journal
https://www.readbyqxmd.com/read/27739921/spectrum-of-autoimmune-diseases-and-systemic-inflammatory-syndromes-in-patients-with-chronic-myelomonocytic-leukemia
#4
Mohammad Faizan Zahid, Terra L Lasho, Christy Finke, Rhett P Ketterling, Naseema Gangat, Curtis A Hanson, Ayalew Tefferi, Mrinal M Patnaik
No abstract text is available yet for this article.
October 14, 2016: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/27733013/dnmt3a-mutations-are-associated-with-inferior-overall-and-leukemia-free-survival-in-chronic-myelomonocytic-leukemia
#5
Mrinal M Patnaik, Daniela Barraco, Terra L Lasho, Christy M Finke, Curtis A Hanson, Rhett P Ketterling, Naseema Gangat, Ayalew Tefferi
DNMT3A mutations are seen in ∼5% of patients with chronic myelomonocytic leukemia (CMML) and thus far, have had an indeterminate prognostic impact on survival. We carried out this study to assess the prognostic impact of DNMT3A mutations on a larger informative cohort of CMML patients (n=261). DNMT3A mutations were seen in 6% (n=16); 56% (n=9) male, with a median age of 64 years. Eighty-one % of DNMT3A mutations were missense, with the Arg882 mutational hot spot accounting for 63% of all changes. Five (31%) patients had an abnormal karyotype whereas concurrent gene mutations (SF3B1/SRSF2/U2AF1-56%, TET2-50%, and ASXL1-25%) were seen in all patients...
October 12, 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27687869/allogeneic-hematopoietic-stem-cell-transplant-in-adult-patients-with-myelodysplastic-syndrome-myeloproliferative-neoplasm-mds-mpn-overlap-syndromes
#6
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
https://www.readbyqxmd.com/read/27662201/number-and-type-of-tet2-mutations-in-chronic-myelomonocytic-leukemia-and-their-clinical-relevance
#7
M M Patnaik, M F Zahid, T L Lasho, C Finke, R L Ketterling, N Gangat, K D Robertson, C A Hanson, A Tefferi
No abstract text is available yet for this article.
September 23, 2016: Blood Cancer Journal
https://www.readbyqxmd.com/read/27562538/acute-leukemia-in-pregnancy-a-single-institution-experience-with-23-patients
#8
Nosha Farhadfar, Sonia Cerquozzi, Michael R Hessenauer, Mark R Litzow, William J Hogan, Louis Letendre, Mrinal M Patnaik, Ayalew Tefferi, Naseema Gangat
Management of acute leukemia during pregnancy presents a considerable challenge. Herein, we review our experience of 23 patients diagnosed with acute leukemia; during pregnancy at the Mayo Clinic between 1962 and 2016. Ten (43.4%), seven (30.4%), and six (26.2%) patients were diagnosed in first, second, and third trimester, respectively. In approximately, 50% (n = 11) therapeutic terminations or spontaneous abortions occurred. Fifty percent (2/4) of patients diagnosed during either first or second trimester who delayed chemotherapy by greater than one week died during induction therapy...
August 26, 2016: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/27516428/somatic-jak2-mutations-and-their-tumor-phenotypes
#9
Ayalew Tefferi
No abstract text is available yet for this article.
August 11, 2016: Blood
https://www.readbyqxmd.com/read/27479179/vascular-events-and-risk-factors-for-thrombosis-in-refractory-anemia-with-ring-sideroblasts-and-thrombocytosis-rars-t
#10
M M Patnaik, T L Lasho, C M Finke, C A Hanson, R L King, R P Ketterling, N Gangat, A Tefferi
Leukemia accepted article preview online, 01 August 2016. doi:10.1038/leu.2016.216.
August 1, 2016: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/27467895/optimization-of-organotin-polymers-for-dielectric-applications
#11
Gregory M Treich, Shamima Nasreen, Arun Mannodi Kanakkithodi, Rui Ma, Mattewos Tefferi, James Flynn, Yang Cao, Rampi Ramprasad, Gregory A Sotzing
Recently, there has been a growing interest in developing wide band gap dielectric materials as the next generation insulators for capacitors, photovoltaic devices, and transistors. Organotin polyesters have shown promise as high dielectric constant, low loss, and high band gap materials. Guided by first-principles calculations from density functional theory (DFT), in line with the emerging codesign concept, the polymer poly(dimethyltin 3,3-dimethylglutarate), p(DMTDMG), was identified as a promising candidate for dielectric applications...
August 24, 2016: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/27431664/characteristics-and-treatment-of-polycythemia-vera-patients-in-clinical-practice-a-multicenter-chart-review-on-1476-individuals-in-germany
#12
Kathleen Jentsch-Ullrich, Judith Eberhardt, Vanja Zeremski, Michael Koehler, Denise Wolleschak, Florian H Heidel
PURPOSE: Treatment recommendations for patients with polycythemia vera (PV) are well established. Most multicenter trials investigating novel therapeutic strategies for PV are developed and conducted at university hospitals and specialized academic centers. The majority of patients in Germany, however, are treated in an outpatient (ambulatory) setting. The aim of this study was to evaluate the 'real-life' situation in a cohort of 1467 patients by analyzing data from a survey conducted at private practices and primary care centers...
September 2016: Journal of Cancer Research and Clinical Oncology
https://www.readbyqxmd.com/read/27407157/allogeneic-hematopoietic-stem-cell-transplantation-for-myelofibrosis-a-practical-review
#13
REVIEW
Nosha Farhadfar, Sonia Cerquozzi, Mrinal Patnaik, Ayalew Tefferi
Myelofibrosis is a myeloproliferative neoplasm with cardinal features of extramedullary hematopoiesis, hepatosplenomegaly, cytopenias, and constitutional symptoms that result in shortened survival and leukemic transformation. It is a disease predominantly of the elderly, and currently available therapies only offer symptom control without curative benefit or ability to alter disease progression. Allogeneic hematopoietic stem-cell transplant (HSCT) is the only potentially curative intervention; however, this is only feasible in younger and medically fit patients and selectively offered to those with high-risk disease...
July 2016: Journal of Oncology Practice
https://www.readbyqxmd.com/read/27353768/bone-marrow-conventional-karyotyping-and-fluorescence-in-situ-hybridization-%C3%A2-defining-an-effective-utilization-strategy-for-evaluation-of-myelodysplastic-syndromes
#14
Rong He, Anne E Wiktor, David K Durnick, Paul J Kurtin, Daniel L Van Dyke, Ayalew Tefferi, Mrinal S Patnaik, Rhett P Ketterling, Curtis A Hanson
OBJECTIVES: The current standard of practice for evaluation of myelodysplastic syndromes (MDS) includes peripheral blood and bone marrow morphology review and conventional karyotyping. Karyotype provides a global view of the chromosome complement while fluorescence in situ hybridization (FISH) targets specific abnormalities. The aim of this study was to determine if an MDS-FISH panel would add value beyond karyotype in MDS workup. METHODS: We studied 505 patients who were evaluated for a possible MDS and had concurrent bone marrow examination, karyotyping, and MDS-FISH performed...
July 2016: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/27341755/the-2016-revision-of-who-classification-of-myeloproliferative-neoplasms-clinical-and-molecular-advances
#15
T Barbui, J Thiele, H Gisslinger, G Finazzi, A M Vannucchi, A Tefferi
Clinical evidence supports the need of changing the diagnostic criteria of the 2008 updated WHO classification for polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). In JAK2-mutated patients who show characteristic bone marrow (BM) morphology, clinical studies demonstrated that a hemoglobin level of 16.5g/dL in men and 16.0g/dl for women or a hematocrit value of 49% in men and 48% in women are the optimal cut off levels for distinguishing JAK2-mutated ET from "masked/prodromal" PV...
June 11, 2016: Blood Reviews
https://www.readbyqxmd.com/read/27315113/leukocytosis-and-presence-of-calr-mutation-is-associated-with-non-hepatosplenic-extramedullary-hematopoiesis-in-primary-myelofibrosis
#16
D Barraco, T L Lasho, N Gangat, C Finke, Y C Elala, A Pardanani, A Tefferi
No abstract text is available yet for this article.
2016: Blood Cancer Journal
https://www.readbyqxmd.com/read/27232929/busulfan-for-the-treatment-of-myeloproliferative-neoplasms-the-mayo-clinic-experience
#17
K Begna, A Abdelatif, S Schwager, C Hanson, A Pardanani, A Tefferi
No abstract text is available yet for this article.
2016: Blood Cancer Journal
https://www.readbyqxmd.com/read/27214377/next-generation-sequencing-in-systemic-mastocytosis-derivation-of-a-mutation-augmented-clinical-prognostic-model-for-survival
#18
Animesh Pardanani, Terra Lasho, Yoseph Elala, Emnet Wassie, Christy Finke, Kaaren K Reichard, Dong Chen, Curtis A Hanson, Rhett P Ketterling, Ayalew Tefferi
In routine practice, the World Health Organization classification of systemic mastocytosis (SM) is also the de facto prognostic system; a core value is distinguishing indolent (ISM) from advanced SM (includes aggressive SM [ASM], SM with associated hematological neoplasm [SM-AHN] and mast cell leukemia [MCL]). We sequenced 27 genes in 150 SM patients to identify mutations that could be integrated into a clinical-molecular prognostic model for survival. Forty four patients (29%) had ISM, 25 (17%) ASM, 80 (53%) SM-AHN and 1 (0...
September 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27185207/chronic-myelomonocytic-leukemia-2016-update-on-diagnosis-risk-stratification-and-management
#19
Mrinal M Patnaik, Ayalew Tefferi
Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder characterized by overlapping features of myelodysplastic syndromes and myeloproliferative neoplasms. Diagnosis is based on the presence of persistent (>3 months) peripheral blood monocytosis (>1 × 10(9) /L), along with bone marrow dysplasia. Clonal cytogenetic abnormalities occur in ∼20-30% of patients, while >90% have gene mutations. Mutations involving TET2 (∼60%), SRSF2 (∼50%), ASXL1 (∼40%), and RAS (∼30%) are frequent; with only ASXL1 mutations negatively impacting overall survival...
June 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27167752/rational-co-design-of-polymer-dielectrics-for-energy-storage
#20
Arun Mannodi-Kanakkithodi, Gregory M Treich, Tran Doan Huan, Rui Ma, Mattewos Tefferi, Yang Cao, Gregory A Sotzing, Rampi Ramprasad
Although traditional materials discovery has historically benefited from intuition-driven experimental approaches and serendipity, computational strategies have risen in prominence and proven to be a powerful complement to experiments in the modern materials research environment. It is illustrated here how one may harness a rational co-design approach-involving synergies between high-throughput computational screening and experimental synthesis and testing-with the example of polymer dielectrics design for electrostatic energy storage applications...
August 2016: Advanced Materials
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