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https://www.readbyqxmd.com/read/28430173/liver-function-test-abnormalities-and-their-clinical-relevance-in-primary-myelofibrosis
#1
D Barraco, M Mudireddy, S Shah, C A Hanson, R P Ketterling, N Gangat, A Pardanani, A Tefferi
No abstract text is available yet for this article.
April 21, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/28419466/the-2016-revised-world-health-organization-definition-of-myelodysplastic-syndrome-with-isolated-del-5q-prognostic-implications-of-single-versus-double-cytogenetic-abnormalities
#2
Mark Gurney, Mrinal M Patnaik, Curtis A Hanson, Mark R Litzow, Aref Al-Kali, Rhett P Ketterling, Ayalew Tefferi, Naseema Gangat
The definition of the myelodysplastic syndrome (MDS) subtype 'MDS with isolated del(5q)' was expanded to include cases with one additional non-chromosome 7 based cytogenetic abnormality in the 2016 revised World Health Organization classification. This study applied the revised definition to a large primary MDS cohort, and evaluated the prognostic impact of the additional cytogenetic abnormality. Seventy-two of 1067 patients (7%) met the 'MDS with isolated del(5q)' criteria, 11 (1%) of whom had an additional cytogenetic abnormality...
April 17, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28370510/socio-economic-burden-of-participation-in-clinical-trials-in-patients-with-myeloproliferative-neoplasms
#3
Swati Goel, Chiara Paoli, Alessandra Iurlo, Arturo Pereira, Fabio Efficace, Tiziano Barbui, Ayalew Tefferi, Alessandro M Vannucchi, Francisco Cervantes
OBJECTIVE: To determine the financial and psycho-social impact of participation in clinical trials of patients with BCR/ABL-negative myeloproliferative neoplasms (MPN). METHODS: An international, observational cross section study was performed in 143 consecutive MPN patients (51% myelofibrosis, 36% polycythemia vera, 13% essential thrombocythemia), 68% from Italy, 17% from USA, and 15% from Spain. RESULTS: Thirty-five percent of patients reported having spent more money during the trial than in previous treatments and 21% having missed more workdays...
March 31, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28370365/monocytosis-in-polycythemia-vera-clinical-and-molecular-correlates
#4
Daniela Barraco, Sonia Cerquozzi, Naseema Gangat, Mrinal M Patnaik, Terra Lasho, Christy Finke, Curtis A Hanson, Rhett P Ketterling, Animesh Pardanani, Ayalew Tefferi
Monocytosis (absolute monocyte count, AMC ≥1 x 10(9) /L) might accompany a spectrum of myeloid neoplasms, other than chronic myelomonocytic leukemia (CMML). In the current study, we examined the prevalence, laboratory and molecular correlates, and prognostic relevance of monocytosis in polycythemia vera (PV). Among 267 consecutive patients with World Health Organization (WHO)-defined PV, 55 (21%) patients displayed an AMC of ≥1 x 10(9) /L and 18 (7%) an AMC of ≥1.5 x 10(9) /L. In general, PV patients with monocytosis were significantly older and displayed higher frequencies of leukocytosis (81% vs 50% at AMC ≥1 x 10(9) /L) and TET2/SRSF2 mutations (57%/29% vs 19%/1% at AMC≥1...
March 28, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28362440/prognostic-relevance-of-lymphocytopenia-monocytopenia-and-lymphocyte-to-monocyte-ratio-in-primary-myelodysplastic-syndromes-a-single-center-experience-in-889-patients
#5
L Saeed, M M Patnaik, K H Begna, A Al-Kali, M R Litzow, C A Hanson, R P Ketterling, L F Porrata, A Pardanani, N Gangat, A Tefferi
Current prognostic models for myelodysplastic syndromes (MDS), including the Revised International Prognostic Scoring System (IPSS-R), do not account for host immunity. We retrospectively examined the prognostic relevance of monocytopenia, lymphocytopenia and lymphocyte-to-monocyte ratio (LMR) in a cohort of 889 patients with primary MDS. After a median follow-up of 27 months, 712 (80%) deaths and 116 (13%) leukemic transformation were documented. In univariate analysis, subnormal absolute lymphocyte count (ALC) <0...
March 31, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/28314085/targeted-next-generation-sequencing-and-identification-of-risk-factors-in-world-health-organization-defined-atypical-chronic-myeloid-leukemia
#6
Mrinal M Patnaik, Daniela Barraco, Terra L Lasho, Christy M Finke, Kaaren Reichard, Katherine P Hoversten, Rhett P Ketterling, Naseema Gangat, Ayalew Tefferi
Atypical chronic myeloid leukemia (aCML) is an aggressive myeloid neoplasm with overlapping features of myelodysplastic syndromes (prominent granulocytic dysplasia) and myeloproliferative neoplasms (neutrophilic leukocytosis). We studied 25 molecularly-annotated and World Health Organization defined aCML patients; median age 70 years, 84% males. Cytogenetic abnormalities were seen in 36% and gene mutations in 100%. Mutational frequencies were, ASXL1 28%, TET2 16%, NRAS 16%, SETBP1 12%, RUNX1 12%, ETNK1 8% and PTPN11 4%...
March 17, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28282034/prognostic-impact-of-bone-marrow-fibrosis-in-polycythemia-vera-validation-of-the-iwg-mrt-study-and-additional-observations
#7
D Barraco, S Cerquozzi, C A Hanson, R P Ketterling, A Pardanani, N Gangat, A Tefferi
In 2012, the International Working Group for Myeloproliferative Neoplasms (MPN) Research and Treatment (IWG-MRT) reported an associations between mild bone marrow (BM) fibrosis (⩾grade 1) in polycythemia vera (PV) and a lower incidence of thrombosis during the clinical course and a higher risk of fibrotic progression. The objective in the current study of 262 patients with PV was to validate these observations and also identify other risk factors for myelofibrosis-free survival (MFFS). About 127 (48%) patients displayed ⩾grade 1 reticulin fibrosis at the time of diagnosis; presenting clinical and laboratory features were not significantly different between patients with or without BM fibrosis...
March 10, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/28240607/downregulation-of-gata1-drives-impaired-hematopoiesis-in-primary-myelofibrosis
#8
Laure Gilles, Ahmet Dirim Arslan, Christian Marinaccio, Qiang Jeremy Wen, Priyanka Arya, Maureen McNulty, Qiong Yang, Jonathan C Zhao, Katerina Konstantinoff, Terra Lasho, Animesh Pardanani, Brady Stein, Isabelle Plo, Sriram Sundaravel, Amittha Wickrema, Annarita Migliaccio, Sandeep Gurbuxani, William Vainchenker, Leonidas C Platanias, Ayalew Tefferi, John D Crispino
Primary myelofibrosis (PMF) is a clonal hematologic malignancy characterized by BM fibrosis, extramedullary hematopoiesis, circulating CD34+ cells, splenomegaly, and a propensity to evolve to acute myeloid leukemia. Moreover, the spleen and BM of patients harbor atypical, clustered megakaryocytes, which contribute to the disease by secreting profibrotic cytokines. Here, we have revealed that megakaryocytes in PMF show impaired maturation that is associated with reduced GATA1 protein. In investigating the cause of GATA1 downregulation, our gene-expression study revealed the presence of the RPS14-deficient gene signature, which is associated with defective ribosomal protein function and linked to the erythroid lineage in 5q deletion myelodysplastic syndrome...
April 3, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28211153/the-prognostic-relevance-of-serum-lactate-dehydrogenase-and-mild-bone-marrow-reticulin-fibrosis-in-essential-thrombocythemia
#9
Mythri Mudireddy, Daniela Barraco, Curtis A Hanson, Animesh Pardanani, Naseema Gangat, Ayalew Tefferi
The 2016 World Health Organization (WHO) diagnostic criteria for myeloproliferative neoplasms (MPN) underscore the prognostically-relevant distinction between essential thrombocythemia (ET) and prefibrotic primary myelofibrosis (pre-PMF). In addition, leukocytosis has been identified as an important prognostic marker in otherwise WHO-defined ET. However, controversy remains regarding the objectivity of morphologic criteria in distinguishing ET from pre-PMF and the precise prognostic cutoff values for leukocytosis...
May 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28196400/diagnostic-impact-of-the-2016-revised-who-criteria-for-polycythemia-vera
#10
Tiziano Barbui, Juergen Thiele, Heinz Gisslinger, Alessandra Carobbio, Alessandro M Vannucchi, Ayalew Tefferi
No abstract text is available yet for this article.
May 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28188970/refractory-anemia-with-ring-sideroblasts-rars-and-rars-with-thrombocytosis-rars-t-2017-update-on-diagnosis-risk-stratification-and-management
#11
Mrinal M Patnaik, Ayalew Tefferi
DISEASE OVERVIEW: Ring sideroblasts (RS) are erythroid precursors with abnormal perinuclear mitochondrial iron accumulation. Two myeloid neoplasms defined by the presence of RS, include refractory anemia with ring sideroblasts (RARS), now classified under myelodysplastic syndromes with RS (MDS-RS) and RARS with thrombocytosis (RARS-T); now called myelodysplastic/myeloproliferative neoplasm with RS and thrombocytosis (MDS/MPN-RS-T). DIAGNOSIS: MDS-RS is a lower risk MDS, with single or multilineage dysplasia (SLD/MLD), <5% bone marrow (BM) blasts and ≥15% BM RS (≥5% in the presence of SF3B1 mutations)...
March 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/27991718/polycythemia-vera-and-essential-thrombocythemia-2017-update-on-diagnosis-risk-stratification-and-management
#12
Ayalew Tefferi, Tiziano Barbui
DISEASE OVERVIEW: Polycythemia Vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms respectively characterized by erythrocytosis and thrombocytosis; other disease features include leukocytosis, splenomegaly, thrombosis, bleeding, microcirculatory symptoms, pruritus, and risk of leukemic or fibrotic transformation. DIAGNOSIS: PV is defined by a JAK2 mutation, whose absence, combined with normal or increased serum erythropoietin level, makes the diagnosis unlikely...
January 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/27870387/primary-myelofibrosis-2017-update-on-diagnosis-risk-stratification-and-management
#13
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. The presence of JAK2, CALR or MPL mutation is supportive but not essential for diagnosis; approximately 90% of patients carry one of these mutations and 10% are "triple-negative...
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
#14
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. The objective of this study was to determine the rates of RBC-transfusion independence after therapy with pomalidomide vs placebo in persons with MPN-associated myelofibrosis and RBC-transfusion dependence. Two hundred and fifty-two subjects (intent-to-treat (ITT) population) including 229 subjects confirmed by central review (modified ITT population) were randomly assigned (2:1) to pomalidomide or placebo...
April 2017: 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
#15
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
#16
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
#17
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...
January 2017: 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
#18
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
#19
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
#20
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
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