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ISTH Workshop - Valencia Program
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Organized in Partnership with Sociedad Española de Trombosis y Hemostasia (SETH)

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Program

 

 

Download PDF: Workshop Program

 

THURSDAY, September 6, 2018

17:00-21:00 Arrival & Registration

 

 FRIDAY, September 7, 2018

Time Event Speaker
8:00 Registration open
8:45 Welcome Antonio Moscardó, Spain & José Rivera Pozo, Spain

Session 1. Platelet function and laboratory evaluation I Moderator: Antonio Moscardó, Spain
9:00-9:30

Current and emerging approaches for evaluating platelet function: Overview

In this session, participants will get an updated knowledge in platelet function testing, with remarks on the utility of the different procedures in research and clinical settings.

Marco Cattaneo, Italy
9:30-10:00

Performance and evaluation of platelet aggregation and secretion

This presentation will in introduce the utility of lumiaggregometry for the study of inherited platelet disorders; analyze factors that may affect the technique, and approximations for their standardization and discuss real clinical cases and how the lumiaggregometer can help diagnosis.

Antonio Moscardó, Spain,
10:00-10:30

Flow cytometry: A key tool in modern evaluation of platelet function

After this presentation, participants will receive an introduction to flow cytometry, special considerations for platelet function testing and pros and cons with the technique; get an overview of possible agonists and activation markers used for platelet function testing by flow cytometry and the platelet function defects they can potentially detect; get an insight in the current knowledge regarding the impact of different factors of importance for the results (anticoagulants, dilution, platelet count, temperature etc); and get an update regarding the current status and future perspectives for flow cytometry in platelet function testing.

Sofía Ramstrom, Sweden
10:30-11:00

Testing platelet function under flow

In this session participants will get an overview of flow assays to study platelet function, some considerations when planning flow experiments, limitations of the various approaches and some examples of the way these assays have been applied to study platelet function.

Steve Thomas, UK
11:00-11:30 Coffee break 30 min
Session 2. Platelet function and laboratory evaluation II Moderator: Jon Gibbins, UK
11:30-12:00

Platelet receptors and signaling: An update

Participants in this session will gain an understanding of the key platelets receptor families that mediate the regulation of platelets and the role of the balance between activatory and inhibitory receptors in this; appreciate the complexity of the cell signaling networks that these control the actions of platelet receptors; and gain knowledge of the implications of these receptors in thrombotic disease or its prevention.

 

Jon Gibbins, UK
12:00-12:30

Neonatal megakariopoiesis and platelet function

The goal of this session is for participants to know and understand new mechanisms that explain the hyporeactivity of neonatal platelets to physiological agonists. Even though healthy term neonates present compensatory mechanisms that allow them to maintain a global hemostasis preserved, under pathological conditions (prematurity, sepsis),these mechanisms may not be sufficient, thus contributing to the higher risk of hemorrhage and thrombocytopenia during the neonatal life. In this session, we will know the physiological mechanisms that counterbalance this “neonatal platelet hypofunction”.

Francesca Ferrer, Spain
12:30-13:00

Novel approaches in platelet microscopy

In this session participants will be introduced to several forms of advanced fluorescence microscopy and how these techniques can be applied to studying platelets and megakaryocytes. Techniques will include TIRF, super-resolution and light-sheet microscopy.

Steve Thomas, UK
13:00-13:30

Performance and utility of platelet proteomics: a pharmacological perspective

In this session, participants will gain an understanding of the experimental design and utility of platelet clinical proteomics research for biomarker and drug target discovery. They will also learn how to link platelet proteomics and pharmacological studies for drug discovery.

Angel García, Spain
13:30-15:00 Lunch 90 min
Session 3. Old and new inherited disorders of platelet number and function Moderator: José Rivera Pozo, Spain
15:00-15:30

Inherited platelet disorders in 2018

After this presentation, participants will gain a knowledge in the field of Inherited Platelet Disorders (IPD); understand the benefict of mucticentric collaboartion in research in such as rare diseases; and have the opportunity to network  with other experts in IPDs and to get into collaborative project.

José Rivera Pozo, Spain
15:30-16:00

Update on Inherited Thrombocytopenias

In this session, participants will acquire knowledge on the genetic causes of inherited thrombocytopenias; learn to apply a diagnostic algorithm to identify the different forms of thrombocytopenias  with a focus on laboratory tests; and understand the implications for therapeutic management and prognosis.

Paolo Gresele, Italy
16:00-16:30

Gray platelet syndrome and other severe platelet granule disorders

Participants in this session will learn how platelets are secretory cells that contain 3 types of granules which, from high to low abundance, are alpha-granules, dense-granules and lysosomes; platelet function is mediated by the release of these three types of granules upon vascular injury and aberrations in any of the three types lead to inability of platelets to sustain haemostasis, resulting in bleeding; loss of function mutations in NBEAL2 are causal of Grey Platelet Syndrome characterised by bleeding and a lack of platelet alpha-granules; and how we are now starting to understand the role of NBEAL2 in the homeostasis of alpha-granules.

José Antonio Guerrero, UK
16:30-17:00

Platelet disorders due to transcription factors

This lecture will focus on the genetic defects in transcription factors which are involved in patients with inherited bleeding and associated disorders. This will include highlighting the recent advances in novel genes identified in inherited thrombocytopenia and their suggested role in megakaryocyte and platelet function.

Neil Morgan, UK
17:00-17:30 Coffee break 30 min
Session 4. Diagnosis and management of platelet inherited disorders Moderator: Paolo Gresele, Italy
17:30-18:00

The clinical approach to inherited platelet disorders

Participants in this session will learn about the diagnosis of inherited platelet disorders (IPDs) remains challenging; Personal and familial bleeding history is an important aspect of the diagnosis of bleeding disorders, although other conditions may be present (leukemia, aplastic anemia, infections or cardiovascular risk); a streamlined panel of laboratory tests, with consecutive steps of increasing level of complexity is useful to investigate the phenotypic characterization of most IPDs; and the application of high-throughput sequencing has revolutionized the field of genetic diagnosis in IPD and aided in our successful diagnostic process. 

José María Bastida, Spain
18:00-18:30

Bleeding risk and bleeding management in congenital platelet disorders

In this session, participants will develop an understanding of the bleeding risk of inherited platelet disorders (IPD); clarify the relative severity of post-surgical and post-partum haemorrhage in different forms of IPD; acquire knowledge on the prophylactic and therapeutic management and bleeding in IPDs; and learn to deal with IPD-associated bleeding by interactive clinical case presentations.

Paolo Gresele, Italy
18:30-19:00

Molecular diagnosis of congenital platelet disorders in the genomic era

Participants in this session will receive an overview will be provided of the growing list of genes involved in congenital platelet disorders. High throughput sequencing can provide a rapid diagnosis for congenital platelet disorders and methods to achieve this will be discussed with also a focus on the limitations and difficulties. Whole exome and genomes sequencing are also useful in a research setting for gene discovery. The success is strongly dependent on novel statistical methods, functional genetics and international data exchange. The challenge ahead in the genome era is the exploration of non-coding genomes and alternative modes of inheritance and this will be discussed for congenital platelet disorders. 

Kathleen Freson, Belgium
19:00-19:30

Pathogenicity assessment and ethical rules in the genetic testing of bleeding and platelet disorders

After this presentation, participants will gain an understanding of how genome medicine for clinical diagnostics should meet strict criteria related to variant classification and ethics. This will be discussed with a focus on congenital bleeding and platelet disorders and by providing cases and genetic reporting. We will present pathogenicity assignments rules according to ACMG and overview gene and variant curation processes. An introduction will be provided on disease and variant databases and the current novel initiatives in this field (such as the NIH ClinGen Working group for Thrombosis and Haemostasis). 

Kathleen Freson, Belgium
End of sessions
19:30-20:30 Spanish wine and free discussion with experts
20:30 Dinner
 

SATURDAY, September 8, 2018

Time Event Speaker

Session 5. Novel aspects in platelet physiology Moderator: KJ Clemetson, Switzerland
9:00-9:30

Additional roles of platelets in human health and disease

Participants in this presentation will learn that platelets have major roles in haemostasis and thrombosis, which have been known for a long time. Over the past few decades more and more aspects of platelet function have been described or indicated.  Platelets also contribute, to varying extents, to various aspects of immunity, in particular but not only, innate immunity. In addition, platelets have important roles in maintaining the vascular system and, during development, separating it from the lymphatic system. Platelets still contain many receptors and granule components that have poorly defined functions. In investigating patients with bleeding disorders it is important to know if the patients have other mild symptoms that may be due to indirect effects.

KJ Clemetson, Switzerland
9:30-10:00

MicroRNAs in platelet physiology and function

In this presentation, participants learn the basics on microRNA biology; acquire knowledge on the role of miRNAs in platelet physiology and function; learn how dysregulation of platelet miRNAs may provoke thrombosis; and approach the use of platelet miRNAs as biomarkers of cardiovascular disease.

Constantino Martínez, Spain
10:00-10:30

New mechanisms of platelet formation

In this presentation, participants will learn about how small GTPases regulate megakaryocyte localization and demarcation membrane system polarization; rearrangement of the F-actin network and mechanotransduction is important for the maturation and positioning of the demarcation membrane system (DMS), and the initiation of proplatelet formation, whereas dynein-driven microtubule sliding is the primary driving force of proplatelet extension; and GPVI signaling is compromised in newly formed young platelets after acute thrombocytopenia in mice.

Markus Bender, Germany
10:30-11:00

Recent updates on de novo protein synthesis by platelets

Participants in this session will be able to understand the potential of the proteome landscape of platelets to undergo changes upon activation; acquire knowledge on the processes that regulate platelet proteome modifications (e.g. pre-mRNAs splicing); and clarify the possible functional or pathophysiological implications of these processes.

Loredana Bury, Italy
11:00-11:30 Coffee Break (30 min)
Session 6. Acquired disorders of platelet function and number Moderator: Maria Luisa Lozano, Spain
11:30-12:00

Current status in platelet reactivity monitoring and antiplatelet therapy in cardiovascular disease

In this presentation, participants will get an updated perspective of the clinical utility of platelet reactivity monitoring in patients on treatment with antiplatelet therapy.

Marco Cattaneo, Italy
12:00-12:30

Contemporary diagnosis and management of primary  immune thrombocytopenic purpura (ITP)

This presentation will detail the pathophysiology of primary immune thrombocytopenia (ITP). Participants will learn to recognize the limitations of current diagnostic approaches and develop a rational approach to treatment of ITP and be aware of novel therapeutic approaches.

Maria Luisa Lozano, Spain
12:30-13:00

Bleeding and thrombotic complications of acute myeloid leukemia

In this session, participants will learn the incidence, outcome and prognostic factors (in AML/APL) for: coagulopathy, bleeding, and thrombosis; and the current consensus and controversies on their most appropriate management will be discussed.

Pau Montesinos, Spain
13:00-13:30

Heparin-induced thrombocytopenia

After this presentation, participants will be able to summarize the pathogenesis and clinical consequences of HIT: idiosyncratic drug-induced thrombocytopenia associated with thrombosis; describe the diagnostic process; consider the clinical 4T score in HIT patients; and review currently available therapeutic options and describe alternative anticoagulants.

José Antonio Páramo, Spain
13:30-15:00 Lunch 90 min
Session 7. Non-platelet bleeding disorders I Moderator: Teresa Sevivas, Portugal
15:00-15:30

Pathophysiology concepts and novel management of thrombotic microangiopathies

After this presentation, participants will be able to outline the clinical and biologic features that would prompt a clinician to investigate an underlying thrombotic microangiopathy; establish the differential diagnosis of thrombotic; microangiopathies, with emphasis in thrombotic thrombocytopenic purpura and uremic hemolytic syndrome; define the role of ADAMTS-13 in the context of thrombotic microangiopathy; and formulate in a timely fashion an adapted treatment, including the most recent therapeutic alternatives.

José Antonio Páramo, Spain
15:30-16:00

Clinical and Molecular profile of von Willebrand disease

Participants in this session will learn the assessment of the diagnostic difficulties of the von Willebrand disease (VWD) and the role for molecular testing of von Willebrand factor gene (VWF); and analysis of the Spanish experience: The Molecular and Clinical Profile of VWD: Spanish Registry (PCM-EVW-ES) project. 

Javier Batlle, Spain
16:00-16:30

Novel insights into the clinical phenotype and pathophysiology underlying low VWF levels

In this presentation, participants will learn about the first study in patients with low von Willebrand factor levels (30-50 IU/dL). This study is based on an Irish cohort of 126 patients. Further, the presenter will talk about the basic aspect of von Willebrand factor as a molecule.

Sonia Aguila, Ireland
16:30-17:00

Clinical utility of the thrombin generation test 

Participants in this session will be able to recognize its applications to discriminate situations of hypo- and hypercoagulability, but also as it has applications in pathologies where inflammation, the immune system or liver disorders are important.

Silva Navarro, Spain
17:00-17:30 Coffee Break (30 min)
Session 8. Non-platelet bleeding disorders II Moderator: Victor Jimenez Yuste, Spain
17:30-18:00

Hemophilia: Successes and failures until 2018

In this session participants will learn about the issues of hemophilia in 2018. Since hemophilia is a monogenic hereditary disease, it has become a model of this type of alterations. The clinical profile of the disease and the standard of care around the world will be summarized, as well the role of different stakeholders in hemophilia care. From the ancient times to the current time, we will discuss the evolution in the diagnosis and management of this particular bleeding disorder, and will point out the unmet needs that will be reviewed in the following sessions. 

Santiago Bonanad, Spain
18:00-18:30

Novel therapies in hemophilia

In this presentation, participants will learn about the current management options for people with hemophilia, treatment gaps and unmet needs in hemophilia therapy, and novel therapeutics approaches by several different mechanisms.

Victor Jimenez Yuste, Spain
18:30-19:00

Hemophilic arthropathy: pathogenesis, clinical manifestations and evaluation

After this presentation, participants will come away with basic knowledge about the sequelae of repetitive bleedings in musculoskeletal system in subject´s affected by hemophilia. Furthermore, they will able to evaluate the joint damage with radiological and clinical tools to be used in clinical practice.

Sofia Pérez, Spain
19:00-19:30

An overview of coagulation factor defects, other than Hemophilia

In this presentation, participants will learn about rare bleeding disorders (RBDs) and their management is suboptimal; therefore, patients often experience morbidity and mortality due to delayed diagnosis. Clinical symptoms among RBD patients vary significantly between disorders, and patients, even when affected with the same disorder. They will gain the understanding of pathophysiology, presentation, and treatment options for RBDs is critical to facilitate genetic counseling, optimal patient management, and improved long-term outcomes. In addition to that, participants will also learn about expansion and harmonization of international registries has been initiated to correlate genotype, laboratory, and clinical phenotypes including bleeding severity to improve the diagnosis and therapeutic approach.

José María Bastida, Spain

End of Sessions
19:30-20:00 Open debate and concluding remarks Antonio Moscardó & José Rivera
21:00 Dinner and farewell

 



Questions? 
Email education@isth.org.

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