Search results for "polycythemia vera"

Long-Term Survival Data in 652 Patients with Primary Myelofibrosis or Polycythemia Vera— Trends in Recent Years.

2009

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…

A case of severe dermatitis in a patient with Polycythemia Vera during cytoreductive therapy

2019

Polycythemia Vera (PV) is a Philadelphia-negative chronic myeloproliferative neoplasm (MPN) mainly characterized by erythrocytosis. In this report we describe a case of severe cutaneous toxicity in patients with PV treated with hydroxyurea. A 72-year-old woman diagnosed with PV with V617F mutation of JAK2 performed more than 10 years before and treated with hydroxyurea plus phlebotomies and low-dose ASA for about 7 years addressed our center for the appearance of serious dermatitis at the face symptomatic for severe itch. The patient underwent a dermatology visit with diagnosis of desquamative dermatitis due to iatrogenic cause related to the use of hydroxyurea. HU was stopped for a month w…

COMPARISON OF CLINICAL AND LABORATORY DATA, INCLUDING JAK-2 46/1 HAPLOTYPE, BETWEEN PATIENTS WITH IDIOPATHIC ERYTHROCYTOSIS AND POLYCYTHEMIA VERA.

2017

Evaluation of thrombin generation in classical Philadelphianegative myeloproliferative neoplasms / Evaluarea generării trombinei în neoplasmele mielo…

2016

Abstract Introduction: Patients with Philadelphia-negative chronic myeloproliferative neoplasms (Ph-MPN), polycytemia vera (PV), essential thrombocythaemia (ET) and primary myelofibrosis (PMF), are prone to develop thrombotic events. We aimed to investigate the coagulation status in their plasma using thrombin generation assay (TGA), a functional global assay, on Ceveron® Alpha. Materials and methods: The samples were collected from 89 consecutive Ph-negative MPN patients and from 78 controls into K2EDTA and CTAD tubes for blood cell counts, TGA and coagulation screening tests. Thrombin generation was analysed in platelet-poor plasma using Technothrombin® TGA assay kit. Results: We found a …

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…

Thrombin generation - a potentially useful biomarker of thrombotic risk in Philadelphia-negative myeloproliferative neoplasms.

2017

The diagnosis of essential thrombocythemia and polycythemia vera is often made during a thrombotic event which can be serious. Philadelphia-negative chronic myeloproliferative neoplasia patients have an increased thrombotic risk. This is assessed using various scoring systems but these are far from ideal and individual risk. The currend trend to personalised medicine requires finding the most useful thrombotic risk biomarker in these patients. Routine tests for coagulation do not take account of both pro- and anti-coagulant factors which is why these tests are not useful in patients with Philadelphia-negative myeloproliferative neoplasms. Thrombin generation reflects more accurately the bal…

Molecular analysis of the erythropoietin receptor system in patients with polycythaemia vera

1994

Summary Erythropoietin (EPO) is a potent regulator of the viability, proliferation and differentiation of erythroid progenitor cells. Its effect is mediated by binding to the erythropoietin receptor (EPO-R), a member of a new cytokine receptor family. Alterations of the EPO/EPO-R system have recently been shown to be involved in the pathogenesis of familial erythrocytosis and polycythaemia vera (PV). In order to define whether genetic changes in the EPO-R gene and its ligand play a role in the development of PV, the structure and expression levels of the EPO-R and EPO genes were examined in samples from bone marrow and/or peripheral blood mononuclear cells of 24 patients with PV. As expecte…

Morphometric Study of the Bone Marrow in Polycythemia Vera Following Interferon-Alpha Therapy

1993

Bone marrow cellularity and extent of fibrotic change were determined in nineteen patients with polycythemia vera, treated with interferon-alpha (IFN) for 1 year. The cellularity was evaluated with an interactive semiautomatic method using Leitz TAS plus microscope: in particular, number and size of megakaryocytes were evaluated after immunostaining with Y2/51 (CD 61); reticulin content was studied by light microscope with a semiquantitative method. Before IFN therapy mean cellularity was 80.5% (+/- 13.7). After 6 and 12 months mean cellularity was 75.4% and 68.4% respectively. Six months after cessation of IFN therapy the cellularity was 69.1%. A decrease of the number, density and morphom…

Splenomegaly Impacts Prognosis in Essential Thrombocythemia and Polycythemia Vera: A Single Center Study

2019

Splenomegaly is one of the major clinical manifestations of primary myelofibrosis and is common also in other chronic Philadelphia-negative myeloproliferative neoplasms, causing symptoms and signs and affecting quality of life of patients diagnosed with these diseases. We aimed to study the impact that such alteration has on thrombotic risk and on the survival of patients with essential thrombocythemia and patients with Polycythemia Vera (PV). We studied the relationship between splenomegaly (and its grade), thrombosis and survival in 238 patients with et and 165 patients with PV followed at our center between January 1997 and May 2019.

Bone Marrow Biopsy Revision According to WHO Criteria in 272 Patients of the Registro Italiano Trombocitemia (RIT): Preliminary Report On Clinical an…

2009

Abstract Abstract 4974 Background The bone marrow trephine biopsy (BMB) has a crucial role for the diagnosis of essential thrombocythemia (ET), both according to the PVSG and the WHO criteria. The WHO 2001 criteria enhanced the role of BMB also by distinguishing the true-ET (ET) from the prefibrotic and the early fibrotic chronic idiopathic myelofibrosis. The WHO 2008 criteria, in the JAK2 era, confirmed the diagnostic and prognostic relevance of the histopathological features in ET as well as in the other Ph-neg myeloproliferative neoplasms (MPN). Otherwise, only few validated data are presently available, and the reproducibility in the evaluation of some morphological details is still con…