Journal of Hematology & Thromboembolic Diseases
Open Access

Abstract

Myelofibrosis is a Secondary Event in JAK2 Trilinear Myeloproliferative Neoplasm (MPN) and in CALR and MPL Thrombocythemia: Implications for Novel Treatment Options of Prefibrotic MPN

Michiels JJ, Berneman Z, Gadisseur A, Raeve HD, Schroyens W, Potter V, Schelfout K and Valster F

JAK2V617F PV is a trilinear myeloproliferative neoplasm preceded by erythromelalgic thrombocythemia followed by myeloproliferative myeloid metaplasia of spleen and bone marrow and secondary myelofibrosis. The CALR and MPL mutated JAK2 wild type thrombocythemia complicated by myelofibrosis (MF) and agnogenic myeloid metaplasia (AMM) have no features of polycythemia vera (PV) are not primary or agnogenic anymore. The natural history of CALR and MPL thrombocythemia and secondary bone marrow fibrosis clearly differ fromJAK2V617F trilinear essential thrombocythemia (ET), PV, post-ET and post-PV secondary myelofibrosis. Evolution of anemia, splenomegaly and myelofibrosis in MPL, CALR thrombocythemia and JAK2V617F trilinear thrombocythemia and polycythemia vera (TPV) should be evaluated separately simple because treatment options differ.