Search results for "ESSENTIAL THROMBOCYTHEMIA"
showing 10 items of 38 documents
Differential diagnostic features of bone marrow biopsies in essential thrombocythemia
2004
Essential Thrombocythemia (ET) is a chronic myeloproliferative disorder (CMPD) characterized by a high platelet count and originating from a multipotent stem cell. For a long time, according to Polycythaemia Vera Study Group (PVSG) criteria, ET diagnosis has not included histopathological data. Bone Marrow (BM) histology was used only to exclude previous or other subtypes of Ph-CMD or Myelodysplastic syndromes (MDS). In addition, the lack of any cytogenetic or molecular-biological marker has made the discrimination between ET and cases of Reactive Thrombocytosys (RT) without a well known cause quite problematic. Analogously, the distinction of ET from the other Ph- CMPDs with similar clinic…
Excess Mortality in Polycythemia Vera and Essential Thrombocythemia
2018
Abstract Background and objective. An important proportion of patients with polycythemia vera (PV) and essential thrombocythemia (ET) are diagnosed in the seventh and eighth decades of life. Because of the chronic course of PV and ET and the advanced age of patients, many will actually die from age-related ailments instead of the myeloproliferative neoplasm, so the disease's impact on life-expectancy remains largely unknown. This registry‐based study was aimed at investigating the excess mortality attributable to PV and ET in a large series of patients diagnosed and managed according to modern criteria. Methods. We queried the databases of the Spanish Group for Chronic Myeloproliferative Ne…
Comparison between thrombotic risk scores in essential thrombocythemia and survival implications.
2019
The conventional thrombotic risk stratification in essential thrombocythemia (ET) distinguishes patients in two risk groups based on previous thrombosis and age (< or >60). The IPSET-thrombosis takes into account four risk factors: age greater than 60 years and the presence of CV risk factors, thrombosis history and JAK2 V617F presence. The revised IPSET-thrombosis uses three adverse variables to delineate four risk categories: age greater than 60, thrombosis history, and JAK2 V617F presence. We compared different risk models in the estimation of thrombotic risk in 191 patients with ET and the role of specific driver mutations affecting overall survival, according to thrombotic risk. …
Presence of calreticulin mutations in JAK2-negative polycythemia vera
2014
Abstract Calreticulin (CALR) mutations have recently been reported in JAK2- and MPL-negative Myeloproliferative Neoplasms (MPN), particularly essential thrombocythemia (ET) and primary myelofibrosis (PMF).The clinical course of sporadic CALR-mutated patients seems to be more indolent than that of JAK2-mutated patients. In contrast, no CALR mutation has been found in the 647 published cases of Polycythemia Vera (PV) patients tested. Consequently, CALR mutations were considered exclusive to JAK2 and MPL mutations. Since 98% of PV patients harbor a JAK2 mutation (mostly the V617F mutation in exon 14 and more rarely, in exon 12), the absence of CALR mutations in PV seemed logical. Here, we desc…
Cardiovascular events and intensity of treatment in polycythemia vera.
2013
A b s t r ac t Background Current treatment recommendations for patients with polycythemia vera call for maintaining a hematocrit of less than 45%, but this therapeutic strategy has not been tested in a randomized clinical trial. Methods We randomly assigned 365 adults with JAK2-positive polycythemia vera who were being treated with phlebotomy, hydroxyurea, or both to receive either more intensive treatment (target hematocrit, <45%) (low-hematocrit group) or less intensive treatment (target hematocrit, 45 to 50%) (high-hematocrit group). The primary composite end point was the time until death from cardiovascular causes or major thrombotic events. The secondary end points were cardiovascula…
Survival of European patients diagnosed with myeloid malignancies: a HAEMACARE study
2013
Population-based information on the survival of patients with myeloid malignancies is rare mainly because some entities were not recognized as malignant until the publication of the third revision of the International Classification of Diseases for Oncology and World Health Organization classification in 2000. In this study we report the survival of patients with myeloid malignancies, classified by updated criteria, in Europe. We analyzed 58,800 cases incident between 1995 to 2002 in 48 population-based cancer registries from 20 European countries, classified into HAEMACARE myeloid malignancy groupings. The period approach was used to estimate 5-year relative survival in 2000-2002. The rela…
Frequent reduction or absence of detection of the JAK2-mutated clone in JAK2V617F-positive patients within the first years of hydroxyurea therapy
2008
Abstract Objective: To analyse the effect of hydroxyurea (HU) on the JAK2-V617F allelic ratio (%JAK2-V617F) of patients with Polycythaemia Vera (PV) and Essential Thrombocythaemia (ET). Methods: Thirty-six patients were examined sequentially prior to and after on-set of (HU) therapy (8 PV, 17 ET), or while remaining untreated (2 PV, 9 ET). For all patients, the %JAK2-V617F was determined in purified blood granulocytes using sensitive allele-specific, quantitative PCRs. In a second study, two distinct groups of patients were examined at a single time point at the time of diagnosis (99 PV, 178 ET) or while receiving HU (36 PV, 98 ET). Results: HU therapy (median duration: 15 months) reduced t…
Correlation between leukocytosis and thrombosis in Philadelphia-negative chronic myeloproliferative neoplasms
2009
The evidence that leukocytes may contribute to the pathogenesis of thrombosis in Chronic Myeloproliferative Neoplasms is increasing but not definitive. To further enforces whether an increased leukocyte count is associated with thrombosis and whether this effect can be modulated by cytoreductive therapy, we analyzed the clinical course of 187 patients with Polycythemia Vera (PV) and Essential Thrombocythemia (ET) followed at two Italian Institutions over a period of 7 years. The association was measured at diagnosis or before thrombotic events: a multivariable analysis was carried out using data at baseline and time-dependent covariates. We found that white blood cells (WBC) count above 9.5…
Essential thrombocythemia terminating in pure erythroleukemia
2004
Transformation into acute leukemia is a rare event in essential thrombocythemia (ET). The blasts are usually of myeloid, rarely of megakaryoblastic differentiation. We present the case of a patient with pure erythroleukemia after a nearly 10-year course of ET, which was treated with hydroxyurea. The patient, a 58-year-old male, presented with an elevated thrombocyte count (926,000/μL) and normal values of hemoglobin and leukocytes. After 10 years of therapy with hydroxyurea, the patient developed acute leukemia of solely erythroid differentiation. Chemotherapy with cytarabine and daunorubicin resulted in incomplete remission. The patient died 2 months after diagnosis of acute erythroleukemi…
Chromosome 1 abnormalities in myeloid malignancies: a literature survey and karyotype-phenotype associations.
2009
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…