Abstract In the 2016 revised classification of myeloproliferative neoplasms pre-fibrotic primary myelofibrosis (pre-PMF) was recognized as a separate entity, distinct from essential thrombocythemia (ET). Owing that the majority of cases falling in the pre-PMF category were previously diagnosed as ET, one may question about the need to re-evaluate the results of epidemiologic, clinical, and molecular […]
Imetelstat Active in Myeloproliferative Disorders
Essential thrombocythemia, myelofibrosis respond to telomere targeting Treatment with a drug that targets the chromosome-lengthening enzyme telomerase led to hematologic responses in all treated patients with essential thrombocythemia, a preliminary clinical trial showed. In 16 of 18 cases, patients achieved complete hematologic responses with imetelstat. Additionally, seven of eight patients with Janus kinase 2 (JAK2) […]
CALR Mutated Essential Thrombocythemia is a distinct disease
JAK2 or CALR mutation status defines subtypes of ET with substantially different clinical course and outcomes Key Points JAK2 (V617F) mutated essential thrombocythemia and polycythemia vera are different phenotypes in the evolution of a single neoplasm CALR mutated essential thrombocythemia is a distinct disease entity not only at molecular level but also with respect to clinical outcomes Abstract Patients with […]
