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Global Hematology 2019 Hematology and Blood Disorders | Volume 2
Page 14
July 25-26, 2019 | Amsterdam, Netherlands
OF EXCELLENCE
IN INTERNATIONAL
MEETINGS
YEARS
4th International Conference on
HEMATOLOGY AND
BONE MARROW TRANSPLANTATION
TRANSLATION OF PVSG/WHO INTO THE
CLINICAL, LABORATORY, MOLECULAR AND
PATHOLOGICAL (2018 CLMP) DEFINED
MYELOPROLIFERATIVE NEOPLASMS CAUSED BY
JAK2V617F JAK2EXON12, CALR, MPL AND TPO
DRIVER MUTATIONS ARE DISTINCT BLOOD AND
COAGULATION DISORDERS: PROGNOSTIC AND
THERAPEUTIC IMPLICATIONS TOWARDS 2020
AND BEYOND
The JAK2V617F mutated tri-linear myeloproliferative neoplasms (MPN) in-
clude a broad spectrum of clinical laboratory and bone marrow features
in essential thrombocythaemia (ET), prodromal polycythaemia vera (PV) and
erythrocythemic PV, classical PV and advanced stages of masked PV and PV
complicated by splenomegaly and secondary myelofibrosis (MF). Hetero-
zygous JAK2V617F mutated ET is associated with low JAK2 allele and MPN
disease burden and normal life expectance. In combined heterozygous and
homozygous or homozygous JAK2V617F mutated tri-linear MPN, the JAK2
mutation load increases from less than 50% in prodromal and early stage PV
to above 50% up to 100%in classical PV, advanced PV and PV with MF. Bone
marrow histology features show various degrees of diagnostic erythrocytic,
megakaryocytic and granulocytic (EMG) myeloproliferation in JAK2V617F
mutated tri-linear MPN clearly differ frommono-linear megakaryocytic (M) in
MPL or dual megakaryocytic granulocytic (MG) myeloproliferation in calretic-
ulin (CALR) mutated thrombocythemia without features of PV. The morphol-
ogy of clustered large pleomorphic megakaryocytes with hyper lobulated
nuclei is similar in JAK2V67F thrombocythemia, prodromal PV and classical
PV patients. Mono-linear megakaryocytic (M) myeloproliferation of large
to giant megakaryocytes with hyper lobulated staghorn like nuclei is the
hallmark of MPL515 mutated normocellular thrombocythaemia. CALR mu-
tated thrombocythaemia usually presents with high platelet count around
Jan Jacques Michiels, Hematol Blood Disord 2019, Volume 2
Jan Jacques Michiels Multidisciplinary Internist
Blood Coagulation and Vascular Medicine Center,
Erasmus Tower, Veenmos 13, 3069 AT Rotterdam,
NL. He is Professor of Nature Medicine and Health
Clinical and Molecular Genetics Blood and Coag-
ulation Research, University Hospitals Antwerp,
Brussels and Martin-Bratislava International Con-
sultant Blood coagulation andVascular Medicine,
Consultant Academic of Pharmaceutical and In-
dustrial Medicine. He is Editor of Journal of Hae-
matology & Thromboembolic Diseases, World Jour-
nal of Haematology and Editor in Chief of World
Journal of Clinical Cases.
Jan Jacques Michiels
Goodheart Institute and Foundation in Nature Medicine & Health,
Netherlands
BIOGRAPHY