JAKARTA is a pivotal phase 3, multicenter, randomized, double-blind, placebo-controlled trial evaluating the efficacy of daily oral doses (400 mg or 500 mg) of fedratinib compared with placebo in patients with intermediate-2 or high-risk primary myelofibrosis
, post-polycythemia vera myelofibrosis or post-essential thrombocythemia myelofibrosis with splenomegaly.
Disorders altering the myeloid elements include the following disorders: Polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis
(PMF), chronic myelogenous leukemia (CML), chronic neutrophilic leukemia (CNL), hypereosinophilic leukemia/CEL, CMML, and mast cell disease.
Relative fluorescence intensity of myelofibrosis sections (primary myelofibrosis
and acute monocytic leukemia with reticulin myelofibrosis) without 4',6-diamidino-2-phenylindole (DAPI) staining in 488-, 561-, and 633-nm channels, with that in 405-nm channel used as control.
Thrombospondin-1 (TSP-1) in primary myelofibrosis
(PMF)--A megakaryocyte-derived biomarker which largely discriminates PMF from essential thrombocythemia.
V617F mutation arises in 95% of polycythemia vera, 50-60% of essential thrombocythemia and 30-50% of primary myelofibrosis
patients.4,10,12 Majority of PV patients (95%) show V617F mutation while the remaining cases (5%) of PV exhibits Jak2 gene exon 12 mutation.10
Due to the presence of pleural effusion, in combination with RBBB and respiratory alkalosis in a patient with underlying procoagulant condition (primary myelofibrosis
), a computerized tomography of pulmonary vessels was performed, which excluded the presence of pulmonary embolism/thrombosis.
October 2016: Diagnosis of primary myelofibrosis
with transfusion dependent anemia, thrombocytopenia, and leukopenia.
A diagnosis of paediatric primary myelofibrosis
was made on biopsy.
(PMF) is a myeloproliferative neoplasm characterized by abnormal proliferation of megakaryocytes, bone marrow fibrosis, and extramedullary hematopoiesis.
(1) Between 90% and 98% of patients with polycythemia vera and about 50% of patients with essential thrombocythemia (ET) and primary myelofibrosis
(PMF) harbor the JAK2V617F mutation.
In the classical Philadelphia chromosome-negative myeloproliferative neoplasms, several lines of evidence link ASXL1 mutations with BM fibrosis: ASXL1 mutations are associated with a higher degree of BM fibrosis in primary myelofibrosis
; ASXL1 mutations are associated with an increased risk of myelofibrotic transformation in patients with essential thrombocythemia and polycythemia vera ; and ASXL1 mutations are more frequently detected in overt as opposed to prefibrotic primary myelofibrosis
Adam was diagnosed on July 1st of this year with a rare - and terminal - type of blood cancer (primary myelofibrosis