410 research outputs found
Recent advances in diagnosis and treatment of chronic myeloproliferative neoplasms
The Philadelphia chromosome-negative chronic myeloproliferative neoplasms (MPNs) have recently been the focus of tremendous advances in basic knowledge of disease pathophysiology following the recognition of mutations in JAK2 and MPL. These discoveries also led to refinement of the criteria employed for diagnosis. The prognostic roles of the JAK2 V617F mutation and of leukocytosis as independent risk factors for thrombosis, which represents the leading cause of death in patients with polycythemia vera and essential thrombocythemia, are supported by retrospective studies. A new risk stratification approach to the patient with primary myelofibrosis allows clinicians to distinguish categories of patients with significantly different expected survival. Finally, new drugs are currently being tested for MPNs, and molecular discoveries could ultimately lead to the development of a specific targeted therapy. Overall, significant advances in diagnosis, prognostication, and treatment have taken place in the last couple of years in the field of MPNs
Crustal redistribution, crust-mantle recycling and Phanerozoic evolution of the continental crust
Drilling the Seismogenic Zone of an Erosional Convergent Margin: IODP Costa Rica Seismogenesis Project CRISP
The Ligurian Units of Western Tuscany (Northern Apennines): insight on the influence of pre-existing weakness zones during ocean closure
Most of the tectonic units cropping out in Western Tuscany are fragments of the Jurassic oceanic crust, ophiolitic successions, overlaid diachronously by Upper Cretaceous-middle Eocene carbonate and siliciclastic flysch successions with their Cenomanian-lower Eocene shalycalcareous basal complexes. These units, so called Ligurian, have been emplaced during the closure of the Ligurian-Piedmont Ocean. Ophiolite bearing debris flows are common in the flysch basins and their relationship with ophiolitic tectonic slices points to a strong relation between tectonics and sedimentation from the early compressive events of the Late Cretaceous. The tectonic activity reflects in a rough morphology of the ocean floor. It progressively influences the distribution and sedimentology of the turbidites. During middle Eocene this relationship begun very important and a paleogeographic reconstruction with prominent linear ophiolitic reliefs that bounded some turbiditic basins can be done. In our reconstruction the sedimenta..
Drilling the seismogenic zone of an Erosional convergent margin: IODP Costa Rica Seismogenesis Project CRISP
Chronic Myeloproliferative Neoplasms: a Collaborative Approach
The classic chronic myeloproliferative neoplasms (MPN) include different entities that pose significant challenges for their optimal diagnosis, treatment and overall management. Polycythemia Vera and Essential Thrombocythemia are the most common among chronic myeloproliferative neoplasms (MPNs); major causes of morbidity and mortality are represented by arterial and venous thrombosis, as well as evolution to myelofibrosis or transformation to acute leukemia. However, survival is only minimally affected. Therapy aims at reducing the rate of thrombosis without increasing the risk of hematologic transformation which could be caused by exposure to cytotoxic drugs. On the other hand, survival is significantly reduced in primary myelofibrosis, and the clinical manifestations may be disabling. In the absence of therapies with the potential of curing the disease, a careful risk-oriented approach is employed for stratifying patients to the most appropriate, currently available, therapeutic options. In this brief review, we will discuss some of the key issues that can arise along the clinical course of MPNs and require an integrated, strictly patient-oriented, approach
JAK2 Allele Burden in the Myeloproliferative Neoplasms: Effects on Phenotype, Prognosis and Change with Treatment.
The field of Philadelphia-chromosome-negative chronic myeloproliferative neoplasms (MPNs) has recently witnessed tremendous advances in the basic knowledge of disease pathophysiology that followed the identification of mutations in JAK2 and MPL. These discoveries led to a revision of the criteria employed for diagnosis by the World Health Organization. The prognostic role of the JAK2V617F mutation and of its allelic burden has been the objective of intensive research using a variety of cellular and animal models as well as in large series of patients. While a definitive position cannot yet been taken on all of the issues, there is a consensus that the presence of higher V617F allele burden, that is on the basis of a stronger activation of intracellular signalling pathways, is associated with the clinical phenotype of polycythemia vera and with defined haematological and clinical markers indicative of a more aggressive phenotype. On the other hand, a low allele burden in myelofibrosis is associated with reduced survival. Finally, a significant reduction of JAK2 V617F allele burden has been demonstrated in patients treated with interferon, while the effects of novel JAK1 and JAK2 inhibitors have not yet been fully ascertained
seamount chain subduction zone interactions implications for accretionary and erosive subduction zone behavior
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