Résumé
Objective:To explore the driver mutations in patients with classical myeloproliferative neoplasms (MPN) and their relationships with clinical characteristics.Methods:The clinical data of 186 patients with classical MPN in the Affiliated Yuebei People's Hospital of Shantou University Medical College from January 2013 to October 2019 who met the World Health Organization 2016 MPN diagnostic criteria were retrospectively analyzed. The mutations of diver genes JAK2, CALR and MPL and clinical characteristics, such as white blood cell count, hemoglobin, and platelet count in patients with essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF) were analyzed.Results:Among the 186 MPN patients, 100 were male and 86 were female, with a median age of onset of 62.0 years old (24.0-93.0 years old). There were 125 patients (67.20%) with ET, 44 patients (23.66%) with PV, and 17 patients (9.14%) with PMF. The JAK2V617F mutation was found in 133 patients (71.51%, 133/186). The JAK2V617F mutation rates in patients with ET, PV and PMF were 66.40% (83/125), 88.64% (39/44) and 64.71% (11/17), respectively. Two patients (1.08%, 2/186) with PV were found with JAK2 exon 12 mutation. The CALR exon 9 mutation was found in 32 patients (17.20%, 32/186), with the CALR mutation rates of 24.00% (30/125), 0 and 11.76% (2/17) in patients with ET, PV and PMF, respectively. The MPL exon 10 mutation was found in one ET patient (0.54%, 1/186). CALR mutated ET patients showed higher platelet count [(1 155±537)×10 9/L vs. (997±330)×10 9/L, t = -2.095, P = 0.038], and lower leukocyte count ( t = 2.434, P = 0.017) and hemoglobin ( t = 3.087, P = 0.003) than JAK2V617F mutated ET patients. Two cases of MPN had rare concurrent driver mutations, of which one ET patient with JAK2V617F and CALR mutations and one PMF patient with JAK2V617F and MPLW515L mutations. Conclusions:The detection result of driver mutations is an important basis for precision health care for MPN. Different types of MPN have different detection rates of driver mutations, which are of great significances for judging the clinical characteristics of patients.
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Objective To investigate the effect of idarubiein to imensify the MAC (IMAC) as preparative regimen for autologous peripheral blood stem cell transplantation(APBSCT)in acute myeioid leukemia.Methods Fourteen patients with acute myeloid leukemia who underwent APBSCT were analysed. IMAC was used as preparative regimens.Results All patients were engrafted successfully.The disease-free survival could be Ben in 8 cases(57.1%),the median disease-free survival duration were 26(8-72)months and no treatment-related mortality was present.Conclusion This study suggested that addition of Idarubicin to the MAC preparative regimens Was safe,effective and feasible for patients with acute myeloid leukemia,and may improve disease-free survival and overall survival.
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AIM: To examine whether calcitonin gene-related peptide (CGRP) enhances nitric oxide (NO) level in pulmonary circulation blood and observe the influence of CGRP on mean pulmonary artery pressure (mPAP) in rabbits with acute lung injury (ALI) caused by oleic acid. METHODS: The level of NO was assessed by measuring the presence of nitrite in cervical artery blood by the Griess reaction, mPAP was measured with right ventricular catheter. RESULTS: The level of nitrite in cervical artery blood was significantly increased and the mPAP was markedly reduced after administration of CGRP intravenousely.CONCLUSION: CGRP enhanced the NO level of pulmonary circulation blood and reduces the mPAP significantly in rabbits with ALI.