Myelofibrosis (MF) is a rare disorder that classified as one of the myeloproliferative disorders. It BCR-ABL1- negative neoplasm characterized by abnormal proliferation hemopoietic stem cells within bone marrow, which leads to overproduction fibrous tissue. Our patient young girl 17 year old presented with lump in left upper abdomen, shortness breath & generalized swelling. The diagnosis wa...

Hematological abnormalities are very common in the course of systemic lupus erythematosus (SLE). Myelofibrosis is a bone marrow disorder in which there is excessive fibrous tissue formation in the bone marrow. Various benign and malignant disorders can cause or be associated with a diffuse increase in the bone marrow reticular tissue. Some diseases such as infections, neoplasms, and autoimmune ...

We studied non-driver mutations in 62 subjects with myeloproliferative neoplasm (MPN)-associated myelofibrosis upon diagnosis, including 45 subjects with primary myelofibrosis (PMF) and 17 with post-polycythemia vera or post-essential thrombocythemia myelofibrosis (post-PV/ET MF). Fifty-eight subjects had ≥1 non-driver mutation upon diagnosis. Mutations in mRNA splicing genes, especially in U2A...

Several lines of evidence indicate that the megakaryocyte/platelet lineage is crucial in myelofibrosis induction. The demonstration that NOD/SCID mice with functionally deficient monocytes do not develop fibrotic changes when exposed to thrombopoietin (TPO) also suggests an important role for monocyte/macrophages. However, in this animal model, the development of myelofibrosis is dependent on t...

Myelofibrosis is a slowly evolving pathology, characterized by increased myeloid cells and structural abnormality of the bone marrow matrix, which at end-stage manifests in excessive deposition of reticulin fibers and cross-linked collagen in the bone marrow, suppression of normal hematopoiesis and bone marrow failure. Clinically, myelofibrosis occurs secondary to a variety of hematologic condi...

Hemopoietic progenitor cells (HPC) from myeloproliferative neoplasms (MPN) such as myelofibrosis commonly express mutant JAK2-V617F or other mutations that are associated with increased activities of JAK-STAT5/3, RAS/RAF/MAPK, and PI3K/AKT/mTOR pathways. This confers proliferative and survival advantage on the MPN HPCs. Treatment with JAK tyrosine kinase inhibitor (TKI), for example, TG101209, ...

Idiopathic myelofibrosis (IM) is a disease characterized by marrow fibrosis, abnormal stem/progenitor cell trafficking, and extramedullary hematopoiesis frequently associated with alterations in megakaryocytes (Mks). Mice harboring genetic alterations in either the extrinsic (ectopic thrombopoietin expression, TPO(high) mice) or intrinsic (hypomorphic GATA-1 mutation, GATA-1(low) mice) control ...

Myelofibrosis and gallbladder carcinoma are both very rare diseases. This case report describes a patient with a history of myelofibrosis and colorectal carcinoma who was diagnosed with colorectal liver metastases. Surgery was performed to remove the metastases, and on site, the gallbladder was removed because of involvement in one of the liver lesions. After pathological examination, a primary...

Folic-acid deficiency may arise in conditions which produce an increased demand for the vitamin. Diseases in this category include acute leukaemia, disseminated carcinomatosis (Girdwood, 1959), myelofibrosis (Chanarin, Mollin, and Anderson, 1958), and haemolytic anaemia (Chanarin, Dacie, and Mollin, 1959). The presence of megaloblastic erythropoiesis in myelofibrosis has been described in only ...

BACKGROUND Essential thrombocythemia is a chronic myeloproliferative disorder; patients with this disorder have a propensity to develop thrombosis, myelofibrosis, and leukemia. DESIGN AND METHODS We studied 605 patients with essential thrombocythemia (follow-up 4596 person-years) with the aim of defining prognostic factors for thrombosis, myelofibrosis, and leukemia during follow-up. RESULT...