Speaker Biography

Jan Jacques Michiels

Goodheart Institute & Foundation in Nature Medicine & Health, Freedom of Science and Education on behave of the International Collaborations and Academic Research in Myeloproliferative Neoplasms, Netherlands

Title: Aspirin Responsive Sticky Platelet Mediated Thrombophilia Caused by Gain of Function Mutations in the Thrombopoietin, JAK2 and MPL Genes in Hereditary and Acquired Essential Thrombocythemia

Jan Jacques Michiels

Dr Jan Jacques Michiels is a Lifestyle Physician and Medical Doctor, MD, educated in Internal Medicine, Hematology, Bloodcoagulation and Vascular Medicine and graduated as PhD at the Erasmus University Medical Center, Rotterdam based on his Thesis Erythromelalgia & Thrombocythemia in 1981. Dr Michiels published more than 400 original manuscripts in peer reviewed international medical journals . Dr Michiels frequently served as Guest Editor and was the Founder and Editor in Chief of Seminars in Vascular Medicine. Dr Michiels is the Founder of the Thrombocythemia Vera Study Group (TVSG) in 1994, the European Working Group on Myeloproliferative Disorders EWG.MPD in 1998 and Myeloproliferative Neoplasms EWG.MPN in 2006 as scientific working groups of the European Hematology Association: EHA. Dr. JJ Michiels is the founder and chair of International Collaboration and Academic Research on Myeloproliferative Neoplasms: ICAR.MPN in 2015 within the HARMONY project of the EHA to collect big data on MPN to improve health and quality of life of MPN patients. Dr Jan Jacques Michiels is the founder and scientific director of the Goodheart Institute & Foundation in Nature Medicine & Health, Rotterdam, The Netherlands, Freedom of Science and Education, Free University Network: FUN Europe. Dr JJ Michiels is co-founder of the Central European Vascular Forum (CEVF) and allied Thrombosis Hemostasis Research Group. Dr Michiels serves as consultant professor in the Hematology and Bloodcoagulation University Hospital Antwerp with special attention to MPN and was co-founder of the VWF-VWD Research Institute of the Hemostasis Thrombosis Laboratory at the Department of Hematology University Hospital, Antwerp. Dr JJ Michiels serves as consultant professor in Hematology and Bloodcoagulation, Comenius University, Bratislava, Slovakia; consultant to the Dutch Society of Internal Medicine and the Ministery of Health Science and Sport and consultant of quality driven Industrial and Pharmaceutical Medicine. Dr JJ Michiels is a editor in chief of 3 Medical Journals and acts as a Guest Editor on request and by self initiation. Dr Michiels is active member of the Belgian, Dutch, German and International Societies on Thrombosis and Haemostasis (ISTH), the European Hematology Association (EHA) and Honory Member of the American Society of Hematology (ASH).


Sticky platelet mediated thrombophiia is featured by aspirin responsive and platelet mediated arteriolar microvas-cular circulation disturbances including erythromelalgia and atypical transient ischemic attacks. The spectrum of aspirin responsive sticky platelet mediated thrombophilia (SPT) in hereditary essential thrombocythemia (HET) due to germline gain of function mutations in the TPO, JAK2 and MPL genes is comparable to the spectrum of SPT in acquired essential thrombocythemia (ET) caused by somatic gain of function mutations JAK2V617F and MPL515. Increase of large platelets in blood smears and large mature megakaryocytes with hyperploid nuclei in a normal cellular bone marrow were diagnostic for autosomal dominant HET and for acquired ET. Evolution of HET and ET into secondary myelofibrosis (MF) belong to the natural history of TPO, JAK2, MPL mutated HET and acquired mutated JAK2V617F and MPL515 mutated acquired ET. In congenital HET caused by heterozygous germline gain of function mutation in the TPO and the JAK2 gene, JAK2V617I and JAK2R564Q, the responses of mutated CD33 and CD34+ cells to TPO are increased, whereas the responses to EPO were normal thereby explaining why HET caused by heterozygous germline TPO and JAK2 mutations are associated with the biological characteristics of ET without PV features. Acquired MPL515 mutated ET has no features of PV, whereas acquired JAK2V617F ET is associated with typical features of PV in blood and bone marrow including low serum EPO and sponta-neous endogenous erythroid colony (EEC) formation. CALR mutated ET and BCR/ABL positive ET are associated with the production of indolent platelet with the absence of sticky platelet mediated thrombophilia and show a rather high tendency of ET evolution into myelofibrosis.