Association of mast cells and bone marrow reticulin fibrosis in patients with bcr-abl negative chronic myeloproliferative neoplasms.
Association of mast cells and bone marrow reticulin fibrosis in patients with bcr-abl negative chronic myeloproliferative neoplasms. Blood Cells Mol Dis. 2020 Mar 05;82:102420 Authors: Keski H Abstract BACKGROUND: We aimed to investigate the association of bone marrow mast cell numbers (MCN) and the degree of reticulin fibrosis in patients with chronic myeloproliferative neoplasms (MPN). METHODS: This was a case-control study that recruited 47 patients who were diagnosed with bcr-abl negative MPN. Thirty patients with lymphoma served as controls. JAK2 mutation was studied and all subjects underwent bone marrow biopsy at the time of diagnosis. Mast and CD34+ cells were counted. Marrow reticulin fiber was graded. RESULTS: Thirty-four patients had essential thrombocythemia (ET), 8 patients had primary myelofibrosis (PMF) and 5 patients had polycythemia vera (PV). Fourteen MPN patients had JAK2, whereas the controls had not. MCN was higher in patients than controls (p = 0.001). There was no significant difference regarding CD34. Reticulin fibrosis was present in 57.4% of MPN patients, whereas there was any in controls. PMF patients had more CD34 + cells than PV and ET. PMF patients had more reticulin fibers compared with other subgroups (p
Conclusions The discovery of JAK2V617F mutation in BCR-ABL1-negative MPNs by four different international cooperative groups in 2005 (2–5) led to significant insights on the pathogenesis of these disorders. In fact, this mutation results in a gain-of-function with activation of cytokine and growth factor receptors, and thus of the downstream JAK-STAT pathway (79, 95–98). The JAK2 point mutation in exon 12, present in a small percentage of patients with PV, is able to induce the MPN phenotype through the same pathogenic mechanism (6, 7). In 2006 the MPLW515L/K was reported in ET and PMF patients (44, 45) and d...
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Myelofibrosis is a chronic myeloproliferative neoplasm that has a heterogenous clinical phenotype and prognosis. Current prognostic models use a variety of clinical and laboratory characteristics to define risk groups whose median survivals can differ by nearly a decade. Prior investigations have suggested a negative prognostic role of absolute monocytosis in this disease.
Conclusion Overall, the increased use of ruxolitinib appears to have come predominantly at the expense of thalidomide and ESAs, while not having a large effect on the first line utilization of hydroxyurea. Teaser The FDA approval of ruxolitinib for intermediate and high-risk myelofibrosis in 2011 changed the therapeutic landscape of the disease. We investigated the first-line treatment choices for MF patients in the pre- and post-ruxolitinib eras and found that the increased use of ruxolitinib has come at the expense of several agents, but has not significantly affected the utilization of hydroxyurea in the first-line setting.
CONCLUSION: XN analyzer has improved blast detection in oncohematologic patients. Operators cannot rely on the blast flag alone but have to consider other flags and hemogram data. In lymphoproliferative disorders, XN analyzer yields less samples with pseudobasophilia. Both analyzers must improve flagging for hairy cell leukemia. PMID: 27981789 [PubMed - as supplied by publisher]
ConclusionXN analyzer has improved blast detection in oncohematologic patients. Operators cannot rely on the blast flag alone but have to consider other flags and hemogram data. In lymphoproliferative disorders, XN analyzer yields less samples with pseudobasophilia. Both analyzers must improve flagging for hairy cell leukemia.
We report a 56-year-old man in whom these two disorders were diagnosed concomitantly. Possible etiopathogenic relationships between both disorders are discussed in this case report. SIMILAR CASES PUBLISHED: 6
Polycythemia Vera (PV) is a chronic myeloproliferative neoplasm which is characterized by clonal proliferation causing the accumulation of morphologically normal red and white blood cells, platelets and their precursors. Bilateral or unilateral sensorineural hearing loss, acute hearing loss and vertigo may be the first presenting findings of PV. The aim of this study was to determine the effect of PV on hearing functions and the frequency of impairment in hearing functions seen in affected patient population.
Polycythemia vera (PV) and essential thrombocythemia (ET) are chronic myeloproliferative neoplasms that can progress to secondary myelofibrosis (SMF). Information on SMF biology and outcome is scant.
Publication date: August 2016 Source:Clinical Lymphoma Myeloma and Leukemia, Volume 16, Supplement Author(s): Carlos Besses, Alberto Alvarez-Larrán Polycythemia vera and essential thrombocythemia (ET) are chronic myeloproliferative neoplasms associated with thrombotic or hemorrhagic complications, and increased risk of transformation to myelofibrosis and acute myeloid leukemia. The main goal of therapy is aimed at preventing vascular events that are the leading cause of morbidity and mortality in these patients. Accordingly, risk stratification is the basis for deciding when to treat a patient with cytoreductive th...