0000000000154714
AUTHOR
Animesh Pardanani
Long-Term Survival Data in 652 Patients with Primary Myelofibrosis or Polycythemia Vera— Trends in Recent Years.
Abstract Abstract 2910 Poster Board II-886 Background: Polycythemia vera (PV) and primary myelofibrosis (PMF) are stem cell-derived myeloproliferative neoplasms characterized clinically by increased red cell volume and bone marrow fibrosis, respectively. Phlebotomy is the cornerstone of treatment in PV whereas hydroxyurea has been the traditional drug of choice in both PV and PMF. Over the last 20 years, several new treatment approaches have been introduced and promoted (e.g. interferon-alpha, anagrelide, thalidomide, allogeneic stem cell transplantation) but their benefit in terms of survival has not been confirmed in controlled studies. Methods: Study patients were recruited form the Mayo…
Transfusion-dependency at presentation and its acquisition in the first year of diagnosis are both equally detrimental for survival in primary myelofibrosis - prognostic relevance is independent of IPSS or karyotype
The International Prognostic Scoring System (IPSS) and karyotype are useful tools for risk stratification in primary myelofibrosis (PMF). We examined the additional prognostic impact of red blood cell transfusion need among 254 consecutive patients (median age, 59 years). Sixty-two patients ( approximately 24%) required transfusions at diagnosis whereas 22 ( approximately 9%) became transfusion-dependent and 170 remained transfusion-independent during the first year postdiagnosis; after a median follow-up of 55 months, the respective median survivals were 35, 25, and 117 months (P < 0.01). Multivariable analysis confirmed the IPSS- and karyotype-independent prognostic weight of transfusion …
Refined cytogenetic-risk categorization for overall and leukemia-free survival in primary myelofibrosis: a single center study of 433 patients.
We have previously identified sole +9, 13q- or 20q-, as 'favorable' and sole +8 or complex karyotype as 'unfavorable' cytogenetic abnormalities in primary myelofibrosis (PMF). In this study of 433 PMF patients, we describe additional sole abnormalities with favorable (chromosome 1 translocations/duplications) or unfavorable (-7/7q-) prognosis and also show that other sole or two abnormalities that do not include i(17q), -5/5q-, 12p-, inv(3) or 11q23 rearrangement are prognostically aligned with normal karyotype, which is prognostically favorable. These findings were incorporated into a refined two-tired cytogenetic-risk stratification: unfavorable and favorable karyotype. The respective 5-y…
Transfusion Need at Diagnosis or Its Development During the First Year of Diagnosis in Primary Myelofibrosis: Effect On Survival and Correlation with JAK2 and TET2 Mutational Status.
Abstract Abstract 1909 Poster Board I-932 Background: The International Prognostic Scoring System (IPSS) for primary myelofibrosis (PMF) utilizes five independent predictors of inferior survival; of these, a hemoglobin level <10 g/dL has the highest impact on survival (Cervantes et al. Blood 2009;113:2895). In the current study, we examined the additional prognostic impact of transfusion need at diagnosis or becoming transfusion-dependent in the first year of diagnosis. These events were also correlated with JAK2 or TET2 mutational status. Methods: Patients were selected from the Mayo Clinic PMF database based on availability of bone marrow histology and IPSS-relevant information at diag…
Mature Survival Data for 176 Patients Younger Than 60 Years With Primary Myelofibrosis Diagnosed Between 1976 and 2005: Evidence for Survival Gains in Recent Years
In the past 20 years, management of primary myelofibrosis (PMF) has incorporated new treatment approaches, but survival benefits have not been confirmed in controlled studies. This retrospective study includes 176 consecutive patients younger than age 60 years in whom PMF was diagnosed during a 30-year period (1976-2005). Median age at diagnosis was 50 years (range, 18-59 years), and 98 patients (55%) were men. At the time of this report, 99 patients (56%) had died; the 77 surviving patients were followed up for a median of 8 years (range, 4-24 years). Overall median survival was 9.2 years, and 15- and 20-year survival rates were 32% and 20%, respectively. According to the Dupriez Prognosti…
WHO-defined ‘myelodysplastic syndrome with isolated del(5q)’ in 88 consecutive patients: survival data, leukemic transformation rates and prevalence of JAK2, MPL and IDH mutations
The 2008 World Health Organization (WHO) criteria were used to identify 88 consecutive Mayo Clinic patients with 'myelodysplastic syndrome with isolated del(5q)' (median age 74 years; 60 females). In all, 60 (68%) patients were followed up to the time of their death. Overall median survival was 66 months; leukemic transformation was documented in five (5.7%) cases. Multivariable analysis identified age >or=70 years (P=0.01), transfusion need at diagnosis (P=0.04) and dysgranulopoiesis (P=0.02) as independent predictors of shortened survival; the presence of zero (low risk), one (intermediate risk) or >or=2 (high risk) risk factors corresponded to median survivals of 102, 52 and 27 months, r…
Circulating interleukin (IL)-8, IL-2R, IL-12, and IL-15 levels are independently prognostic in primary myelofibrosis: a comprehensive cytokine profiling study.
Purpose Abnormal cytokine expression accompanies myelofibrosis and might be a therapeutic target for Janus-associated kinase (JAK) inhibitor drugs. This study describes the spectrum of plasma cytokine abnormalities in primary myelofibrosis (PMF) and examines their phenotypic correlates and prognostic significance. Patients and Methods Patients included in this study were required to have archived plasma, bone marrow biopsy, and cytogenetic information available at the time of first referral to the Mayo Clinic. Multiplex biometric sandwich immunoassay was used to measure plasma levels of 30 cytokines. Results In total, 127 PMF patients were studied; comparison with normal controls (n = 35) r…
MPL mutation effect on JAK2 46/1 haplotype frequency in JAK2V617F-negative myeloproliferative neoplasms
MPL mutation effect on JAK2 46/1 haplotype frequency in JAK2 V617F-negative myeloproliferative neoplasms
Chromosome 1 abnormalities in myeloid malignancies: a literature survey and karyotype-phenotype associations.
Chromosome 1 is the largest human chromosome and contains over 1600 known genes and 1000 novel coding sequences or transcripts. It is, therefore, not surprising that recurrent chromosome 1 abnormalities are regularly encountered in both neoplastic and non-neoplastic medical conditions. The current review is focused on myeloid malignancies where we summarize the relevant published literature and discuss specific karyotype-phenotype associations. We show that chromosome 1 abnormalities are most frequent in BCR-ABL-negative classic myeloproliferative neoplasms (MPN): polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). Specific abnormalities include duplicat…