Date: 11th October 2013
Venue: General Hospital Vienna, Austria
On Friday 11st of October the annual meeting 2013 of the Austrian Competence Network on Mastocytosis (AUCNM) was held at the General Hospital Vienna.
The AUCNM is a partner and important element of the European Competence Network on Mastocytosis (ECNM), where a group of clinicians and scientists in Europe establish a network of centers and experts working in the field of mastocytosis.
Mastocytosis is a rare disease characterized by abnormal growth and accumulation of tissue mast cells either in the skin, in internal organs or both the skin and internal organs. Thus, mastocytosis is a primary disease of mast cells and their progenitor cells. The disease was subdivided into cutaneous mastocytosis (CM) and systemic mastocytosis (SM), however now we know several variants and subvariants of mastocytosis. Mast cells in mastocytosis are monoclonal and possess mutations in the KIT proto-oncogene. In the majority of patients with SM the KIT D816V (Asp-816-Val) mutation is found in the bone marrow. Symptoms of mastocytosis can be caused by distribution of proinflammatory mediators (such as histamine) derived from mast cells (so called mediator-associated symptoms) or by aggressive infiltration of organs/tissues by mast cells with subsequent damage of organ function. There are different options to treat mastocytosis. Prophylaxis and avoidance of triggers that cause release of mediators from mast cells, medications that block the release of mediators and cytoreductive therapy.
The aim of the annual meetings is to establish local guidelines for diagnosis and therapy of mastocytosis.
Workshop on Advances in Myeloproliferative Neoplasms
Date: 31st October to 1st November 2013
Venue: Hilton Vienna Danube Waterfront Hotel, Vienna, Austria
In autumn 2013 a workshop on Advances in Myeloproliferative Neoplasms took place in Vienna. The Symposium and Joint Meeting of the ECNM, LBC ONC and SFB F47-B20 was organised by Wolfgang R. Sperr, Emir Hadzijusufovic and Peter Valent. Together with numerous international experts, members of the LBC ONC presented results from the field of fundamental research from molecular genetics to the investigation of mechanisms of neoplastic transformation and the evolution of myeloproliferative diseases.
The first day was focusing on genetic background and somatic aberration profiles on MPN including Ph+ CML, mastocytosis and eosinophil neoplasms. Moreover, mechanisms of transformation and disease evolution with the focus on SETBP1 mutations and genotype-phenotype interactions in JAK2- transformed MPN were presented as well as data about CDK6 as a potential regulator of angiogenesis and cell cycle progression. Also a number of potential drug targets and molecular markers were discussed in the contex of MPN including STAT5.
The second day was focusing on leukemic stem cells in MPN, especially the niche - stem cell interaction and the target expression profiles of LSC in CML and other MPN. The final session was dedicated to mast cell proliferative neoplasm such as aggressive systemic mastocytosis and mast cell leukemia.