Correspondence: Dr. Reetu Baral, MD Department of pathology, KIST Medical College, Lalitpur, Nepal Email: [email protected] Idiopathic Myelofibrosis is an infrequent chronic myeloproliferative disorder characterized by varying degrees of bone marrow fibrosis and extra medullary hematopoiesis, with the fibrosis being a reactive phenomenon to a neoplastic proliferation of a pluripotent hemato...

Myeloproliferative neoplasia (MPN)-associated pulmonary hypertension (PH) is included in group five of the most recent clinical classification of PH.1 The MPNs are a heterogeneous group of disorders that includes disorders with primary expression of a myeloid phenotype and disorders characterized by expression of the Janus Kinase 2 (JAK2) mutation, p.V617F. The latter includes essential thrombo...

Multiple myeloma and JAK2 positive chronic myeloproliferative neoplasms are hematologic malignancies with a completely different cellular origin. Two cases of simultaneous occurrence of multiple myeloma, one with primary myelofibrosis and another one with essential thrombocythemia are reported in this article. In such cases, an accurate diagnosis requires a molecular testing, including gene seq...

Kiladjian JJ, et al. Three-year efficacy, safety , and survival findings from COMFORT-II, a phase 3 study comparing ruxolitinib with best available therapy for myelofibrosis. Impact of mutational status on outcomes in myelofibrosis patients treated with ruxolitinib in the COMFORT-II study. A pooled overall survival analysis of the COMFORT studies: 2 randomized phase 3 trials of ruxolitinib for ...

MMM usually presents with fatigue, weight loss, splenomegaly with or without symptoms. Anemia and various alterations of the white blood cell and/or platelet count are frequently seen at diagnosis. Thrombocytopenia-related bleeding may occur. MMM must be distinguished from myelodysplasia with fibrosis, from acute megakayoblastic leukemia and acute myelofibrosis. As the disease progresses, incre...

Essential thrombocythaemia was first described over 70 years ago. This condition is dominated by thrombotic and haemorrhagic complications and, in the long-term, by risk of transformation to myelofibrosis and/or acute leukaemia. However, it is heterogeneous both clinically and biologically. Here, a review of current concepts in disease aetiology and management is offered with reference to recen...

We have previously shown that mice induced to overexpress thrombopoietin (TPO) by retroviral-mediated gene transfer into bone marrow (BM) cells develop myelofibrosis and osteosclerosis. It was speculated that these effects were secondary to TPO, resulting from high levels of megakaryocytes and platelets. Also, it was proposed that these mice represent a model for myelofibrosis and osteosclerosi...

Dermatomyositis (DM) is a unique inflammatory myopathy with clinical findings of proximal muscle weakness, characteristic rash, and elevated muscle enzymes. The association of DM and malignancy, most commonly adenocarcinoma, is well known. There have been few case reports of primary myelofibrosis associated with DM. We present the case of a 69-year-old male with a history of polycythemia vera (...

grelide in young patients with essential thrombocythemia. Blood 2001;97:863-6. 12 Harrison CN, Campbell PJ, Buck G, Wheatley K et al. A randomised comparison of hydroxyurea and anagrelide in highrisk essential thrombocythemia: the Medical Research Countil PT-1 Trial. N Engl J Med (in press). 13 Tefferi A. Myelofibrosis with myeloid metaplasia. Review. N Engl J Med 2000;342: 1255–65. 14 Tefferi ...