Myeloproliferative Neoplasms

Goal of Therapy and Monitoring the Response in Polycythemia Vera and Essential Thrombocythemia (Specific Issues of Treatment in PV and ET) (Myeloproliferative Neoplasms)

Introduction Polycythemia vera (PV) and essential thrombocythemia (ET) are the most indolent Philadelphianegative myeloproliferative neoplasms (MPN). In fact, several studies have shown that life expectancy in ET is almost similar to that of the general age-and sex-matched population, but life expectancy of PV patients is however significantly altered, especially after the first decade of follow-up […]

First-Line Therapy and Special Issues Management in Polycythemia Vera and Essential Thrombocythemia (Specific Issues of Treatment in PV and ET) (Myeloproliferative Neoplasms) Part 1

Introduction Polycythemia vera (PV) and essential thrombocythemia (ET) are chronic myeloproliferative neoplasms (MPN) characterized by clonal expansion of an abnormal hematopoietic stem/progenitor cell. Natural history of these chronic myeloproliferative neoplasms (MPN) is marked by thrombohemor-rhagic complications and a propensity to transform into myelofibrosis and acute leukemia. Recommendations for management are based on a limited number […]

First-Line Therapy and Special Issues Management in Polycythemia Vera and Essential Thrombocythemia (Specific Issues of Treatment in PV and ET) (Myeloproliferative Neoplasms) Part 2

First-Line Therapy in Special Clinical Situations Special issues in myeloproliferative neoplasms (MPN) comprise clinical conditions with high relevance for the duration and quality of the patient’s life, but with limited evidence to support sound diagnostic and therapeutic recommendations. These issues include MPN in pregnancy, in children, abdominal vein thrombosis, bleeding complications, surgery, and pruritus. Practical […]

Primary and Secondary Antithrombotic Prophylaxis (Specific Issues of Treatment in PV and ET) (Myeloproliferative Neoplasms) Part 1

Introduction Polycythemia vera (PV) and essential thrombo-cythemia (ET) are relatively benign myeloprolif-erative neoplasms (MPNs) and require a treatment strategy mainly aimed at preventing thrombotic complications (Landolfi and Di Gennaro 2011). Over the last three decades, much effort has been devoted at elucidating the pathogenesis of throm-bophilia, at describing their clinical manifestations, as well as at […]

Primary and Secondary Antithrombotic Prophylaxis (Specific Issues of Treatment in PV and ET) (Myeloproliferative Neoplasms) Part 2

Cytoreductive Strategies in Patients with Different Vascular Risk The most appropriate cytoreductive strategy in the individual patient has to be chosen on the basis of the vascular risk. Most young patients with no vascular history have a low or intermediate vascular risk. Patient older than 65 years with three traditional cardiovascular risk factors and patient […]

Polycythemia Vera and Essential Thrombocythemia: When to Change Therapy – Second-Line Options (Specific Issues of Treatment in PV and ET) (Myeloproliferative Neoplasms)

Introduction The management of a patient with polycythemia vera (PV) or essential thrombocythemia (ET) who fails, or develops intolerance to, first-line therapy is an "orphan" field in terms of evidence-based experience as well as number and characteristics of available conventional drugs. On the contrary, it is becoming an "ever-changing" field as concerns novel drugs since, […]

Blastic Transformation of BCR-ABL-Negative Myeloproliferative Neoplasms (Specific Issues of Treatment in PV and ET) Part 1

Abbreviations aCML Atypical chronic myeloid leukemia AML Acute myeloid leukemia ASCT Allogeneic stem cell transplant ASXL1 Additional sex combs-like 1 ATP Adenosine triphosphate CMML Chronic myelomonocytic leukemia CR Complete response ESA Erythroid stimulating agent ET Essential thrombocythemia HDAC Histone deacetylase HPI Hedgehog pathway inhibitors HSCT Hematopoietic stem cell transplant ICSBP Interferon consensus sequence binding protein […]

Blastic Transformation of BCR-ABL-Negative Myeloproliferative Neoplasms (Specific Issues of Treatment in PV and ET) Part 2

Assessing the Importance of Peripheral Blast Percentage Patients with MPNs, particularly PMF and post-ET/PV MF, are predisposed to circulating myelo-blasts in the peripheral blood (Cervantes 2007; Cervantes et al. 2007). This latter phenomenon is true even when there is not a clear increase in bone marrow blast percentage. Reasons for this phenomenon relate to the […]

Current Clinical Needs (Treatment of PMF) (Myeloproliferative Neoplasms)

Introduction Myeloproliferative neoplasms-associated myelofibrosis (MPN-MF) (Mesa et al. 2011a) may arise de novo (primary myelofibrosis, PMF) or may follow clinically overt polycythemia vera (PV) or essential thrombocythemia (ET) (post PV- and post ET-MF). Patients with MPN-MF may be entirely asymptomatic; however, in their majority, MPN-MF patients present a disabling disease that interferes with their quality […]

Risk Stratification in PMF (Treatment of PMF) (Myeloproliferative Neoplasms) Part 1

Primary myelofibrosis (PMF) is a Philadelphianegative myeloproliferative neoplasm (MPN) whose diagnostic criteria have been recently updated.Among MPNs, PMF has the most heterogeneous clinical presentation, which may encompass anemia, splenomegaly, leukocytosis or leukopenia, thrombocytosis or thrombocytopenia, and constitutional symptoms. Median survival in PMF is estimated at 6 years, but it can range from few months to […]

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