Objective: This study compares hemoglobin and erythropoietin levels in patients with polycythemia vera and secondary polycythemia. Study Design: A retrospective cross-sectional study evaluating the serum erythropoieti...Objective: This study compares hemoglobin and erythropoietin levels in patients with polycythemia vera and secondary polycythemia. Study Design: A retrospective cross-sectional study evaluating the serum erythropoietin and hemoglobin levels in patients with polycythemia vera and secondary polycythemia. This study was performed simultaneously in Texas state of the U.S. and Fars Province in Iran. Methods: Hemoglobin, hematocrit and erythropoietin test results were collected from patients aged 19 to 75 years who were diagnosed with polycythemia vera and secondary polycythemia. Patients records with history of thrombocythemia, congestive heart failure, dyspnea, anemia and pregnant woman were excluded from study. Patients in each decade of life were examined in separate groups, so that changes in hemoglobin related to aging did not affect the research results. Results: 75% of the patients were men, and 25% were women. A total of 1580 patients were analyzed in this study. 57.3% of patients in UTMB and 38.8 patients in Iran have hemoglobin level above 17 mg/dl. 74% of patients in UTMB and 88% of patients in Iran have erythropoietin below 10 IU/mL. Polycythemia in UTMB was more common in people over 50 and in Iran in patients under 50 years old. The serum hemoglobin and erythropoietin levels in patients with polycythemia vera were not significantly different in compare to secondary polycythemia patients. Data showed that there were 84 polycythemia patients per 100,000 people. The results of this study in UTMB and Iran showed that 4.5% and 7%, respectively, of patients with polycythemia had a positive JAK2 test. Conclusion: Low erythropoietin levels may not be helpful in differentiating polycythemia vera from secondary polycythemia. .展开更多
Polycythemia vera manifests as a myeloproliferative neoplasm associated with diverse symptoms, including aquagenic pruritis. This systematic review addresses the pressing need to enhance the understanding of the dise...Polycythemia vera manifests as a myeloproliferative neoplasm associated with diverse symptoms, including aquagenic pruritis. This systematic review addresses the pressing need to enhance the understanding of the disease’s symptomatology and optimize treatment strategies for improved patient outcomes. The rarity and low prevalence of polycythemia vera underscore the importance of this investigation, as existing standard of care involves a multifaceted approach and significant healthcare costs. Despite advancement in therapeutic options, persistent symptoms and resistance to first-line treatments pose challenges. Ruxolitinib has emerged as a promising intervention, demonstrating clinically significant improvement for patients. This systematic review appraises three randomized controlled trials, shedding light on the efficacy of ruxolitinib and its potential to ameliorate pruritis symptoms in symptomatic patients.展开更多
BACKGROUND The combination of polycythemia vera(PV)with pathological cardiac hypertro-phy is uncommon.In this study,we describe a case of PV accompanied by pa-thological cardiac hypertrophy.It is hypothesized that the...BACKGROUND The combination of polycythemia vera(PV)with pathological cardiac hypertro-phy is uncommon.In this study,we describe a case of PV accompanied by pa-thological cardiac hypertrophy.It is hypothesized that the pronounced cardiac hypertrophy in this patient has a strong connection with PV.CASE SUMMARY In 2021,a 34-year-old Chinese man experienced chest constriction,shortness of breath,and palpitations during vigorous activity.Each episode lasted several minutes and resolved spontaneously following cessation of vigorous activity.He occasionally experienced syncope and vertigo without a headache.He underwent cardiac magnetic resonance imaging and was diagnosed with“hypertrophic cardiomyopathy(HCM)”.He was discharged after receiving symptomatic treat-ment,which resulted in an improvement.He presented to our department with chest constriction,shortness of breath,and respiratory distress for one month while climbing to the second floor in 2023.His blood pressure was 180/100 mmHg at the time of admittance,and he was receiving antihypertensive treat-ment.He had a history of PV for 2 years without treatment.Symptomatic treat-ment was implemented concurrently with the administration of hydroxyurea upon admission.Good blood pressure control was observed during the long-term follow-up,and echocardiography did not reveal any progression of myocardial hypertrophy.CONCLUSION Clinicians managing PV patients should remain highly vigilant regarding the risks of thrombosis and cardio-vascular complications,particularly in those with refractory hypertension.展开更多
BACKGROUND Patients with deep venous thrombosis(DVT)residing at high altitudes can only rely on anticoagulation therapy,missing the optimal window for surgery or thrombolysis.Concurrently,under these conditions,patien...BACKGROUND Patients with deep venous thrombosis(DVT)residing at high altitudes can only rely on anticoagulation therapy,missing the optimal window for surgery or thrombolysis.Concurrently,under these conditions,patient outcomes can be easily complicated by high-altitude polycythemia(HAPC),which increases the difficulty of treatment and the risk of recurrent thrombosis.To prevent reaching this point,effective screening and targeted interventions are crucial.Thus,this study analyzes and provides a reference for the clinical prediction of thrombosis recurrence in patients with lower-extremity DVT combined with HAPC.AIM To apply the nomogram model in the evaluation of complications in patients with HAPC and DVT who underwent anticoagulation therapy.METHODS A total of 123 patients with HAPC complicated by lower-extremity DVT were followed up for 6-12 months and divided into recurrence and non-recurrence groups according to whether they experienced recurrence of lower-extremity DVT.Clinical data and laboratory indices were compared between the groups to determine the influencing factors of thrombosis recurrence in patients with lowerextremity DVT and HAPC.This study aimed to establish and verify the value of a nomogram model for predicting the risk of thrombus recurrence.RESULTS Logistic regression analysis showed that age,immobilization during follow-up,medication compliance,compliance with wearing elastic stockings,and peripheral blood D-dimer and fibrin degradation product levels were indepen-dent risk factors for thrombosis recurrence in patients with HAPC complicated by DVT.A Hosmer-Lemeshow goodness-of-fit test demonstrated that the nomogram model established based on the results of multivariate logistic regression analysis was effective in predicting the risk of thrombosis recurrence in patients with lowerextremity DVT complicated by HAPC(χ^(2)=0.873;P>0.05).The consistency index of the model was 0.802(95%CI:0.799-0.997),indicating its good accuracy and discrimination.CONCLUSION The column chart model for the personalized prediction of thrombotic recurrence risk has good application value in predicting thrombotic recurrence in patients with lower-limb DVT combined with HAPC after discharge.展开更多
目的通过小样本外显子测序与公用数据库比对进行生物信息挖掘,探寻汉族人群高原红细胞增多症(high altitude polycythemia,HAPC)变异与发病的相关性。方法纳入汉族高原红细胞增多症男性患者4例和高原健康男性5例,采集静脉血并提取DNA进...目的通过小样本外显子测序与公用数据库比对进行生物信息挖掘,探寻汉族人群高原红细胞增多症(high altitude polycythemia,HAPC)变异与发病的相关性。方法纳入汉族高原红细胞增多症男性患者4例和高原健康男性5例,采集静脉血并提取DNA进行全外显子组测序;将测序数据进行功能学富集分析(gene ontology,GO&kyoto encyclopedia of genes and genomes,KEGG)构建突变基因蛋白质互作网络(protein protein interaction,PPI),选择显著性最高的基因,筛选潜在关键HAPC相关变异位点,初步探究相关变异与HAPC发病可能的机制。结果通过全外显子测序,发现HAPC相关的突变基因216个,其中表皮生长因子(epidemal growth farctor,EGF)基因在功能学富集分析(GO&KEGG),蛋白质互作网络(PPI)中具有高度显著性,初步筛选出5个潜在关键HAPC相关变异位点,通过生物信息学预测及分析,最终筛选出EGF基因上3个可能的变异c.2124G>A(p.Met708Ile)、c.2351A>T(p.Asp784Val)以及c.2759A>T(p.Glu920Val),可能是HAPC的突变位点,与HAPC发病相关。结论本研究表明汉族EGF基因的变异与HAPC发病存在相关性。EGF基因的变异可能通过干扰RNA剪接,改变RNA二级结构,影响蛋白质结构,进而影响EGF的生成及HAPC的发生发展。展开更多
Myeloproliferative neoplasms(MPNs)occur due to the abnormal proliferation of one or more terminal myeloid cell lines in peripheral blood.Subjects suffering from MPNs display a high burden of cardiovascular risk factor...Myeloproliferative neoplasms(MPNs)occur due to the abnormal proliferation of one or more terminal myeloid cell lines in peripheral blood.Subjects suffering from MPNs display a high burden of cardiovascular risk factors,and thrombotic events are often the cause of death in this population of patients.Herein,we provide a brief overview of dyslipidemia and metabolic syndrome and their epidemiology in MPNs and examine the common molecular mechanisms between dyslipidemia,metabolic syndrome,and MPNs,with a special focus on cardio-vascular risk,atherosclerosis,and thrombotic events.Furthermore,we investigate the impact of dyslipidemia and metabolic syndrome on the occurrence and survival of thrombosis in MPN patients,as well as the management of dyslipidemia in MPNs,and the impact of MPN treatment on serum lipid concentrations,particularly as side/adverse effects reported in the context of clinical trials.展开更多
文摘Objective: This study compares hemoglobin and erythropoietin levels in patients with polycythemia vera and secondary polycythemia. Study Design: A retrospective cross-sectional study evaluating the serum erythropoietin and hemoglobin levels in patients with polycythemia vera and secondary polycythemia. This study was performed simultaneously in Texas state of the U.S. and Fars Province in Iran. Methods: Hemoglobin, hematocrit and erythropoietin test results were collected from patients aged 19 to 75 years who were diagnosed with polycythemia vera and secondary polycythemia. Patients records with history of thrombocythemia, congestive heart failure, dyspnea, anemia and pregnant woman were excluded from study. Patients in each decade of life were examined in separate groups, so that changes in hemoglobin related to aging did not affect the research results. Results: 75% of the patients were men, and 25% were women. A total of 1580 patients were analyzed in this study. 57.3% of patients in UTMB and 38.8 patients in Iran have hemoglobin level above 17 mg/dl. 74% of patients in UTMB and 88% of patients in Iran have erythropoietin below 10 IU/mL. Polycythemia in UTMB was more common in people over 50 and in Iran in patients under 50 years old. The serum hemoglobin and erythropoietin levels in patients with polycythemia vera were not significantly different in compare to secondary polycythemia patients. Data showed that there were 84 polycythemia patients per 100,000 people. The results of this study in UTMB and Iran showed that 4.5% and 7%, respectively, of patients with polycythemia had a positive JAK2 test. Conclusion: Low erythropoietin levels may not be helpful in differentiating polycythemia vera from secondary polycythemia. .
文摘Polycythemia vera manifests as a myeloproliferative neoplasm associated with diverse symptoms, including aquagenic pruritis. This systematic review addresses the pressing need to enhance the understanding of the disease’s symptomatology and optimize treatment strategies for improved patient outcomes. The rarity and low prevalence of polycythemia vera underscore the importance of this investigation, as existing standard of care involves a multifaceted approach and significant healthcare costs. Despite advancement in therapeutic options, persistent symptoms and resistance to first-line treatments pose challenges. Ruxolitinib has emerged as a promising intervention, demonstrating clinically significant improvement for patients. This systematic review appraises three randomized controlled trials, shedding light on the efficacy of ruxolitinib and its potential to ameliorate pruritis symptoms in symptomatic patients.
基金Supported by Jilin Province Health Research Talent Special Project,No.2020SCZ27.
文摘BACKGROUND The combination of polycythemia vera(PV)with pathological cardiac hypertro-phy is uncommon.In this study,we describe a case of PV accompanied by pa-thological cardiac hypertrophy.It is hypothesized that the pronounced cardiac hypertrophy in this patient has a strong connection with PV.CASE SUMMARY In 2021,a 34-year-old Chinese man experienced chest constriction,shortness of breath,and palpitations during vigorous activity.Each episode lasted several minutes and resolved spontaneously following cessation of vigorous activity.He occasionally experienced syncope and vertigo without a headache.He underwent cardiac magnetic resonance imaging and was diagnosed with“hypertrophic cardiomyopathy(HCM)”.He was discharged after receiving symptomatic treat-ment,which resulted in an improvement.He presented to our department with chest constriction,shortness of breath,and respiratory distress for one month while climbing to the second floor in 2023.His blood pressure was 180/100 mmHg at the time of admittance,and he was receiving antihypertensive treat-ment.He had a history of PV for 2 years without treatment.Symptomatic treat-ment was implemented concurrently with the administration of hydroxyurea upon admission.Good blood pressure control was observed during the long-term follow-up,and echocardiography did not reveal any progression of myocardial hypertrophy.CONCLUSION Clinicians managing PV patients should remain highly vigilant regarding the risks of thrombosis and cardio-vascular complications,particularly in those with refractory hypertension.
基金Supported by Guiding Project of Qinghai Provincial Health Commission,No.2021-wjzdx-89.
文摘BACKGROUND Patients with deep venous thrombosis(DVT)residing at high altitudes can only rely on anticoagulation therapy,missing the optimal window for surgery or thrombolysis.Concurrently,under these conditions,patient outcomes can be easily complicated by high-altitude polycythemia(HAPC),which increases the difficulty of treatment and the risk of recurrent thrombosis.To prevent reaching this point,effective screening and targeted interventions are crucial.Thus,this study analyzes and provides a reference for the clinical prediction of thrombosis recurrence in patients with lower-extremity DVT combined with HAPC.AIM To apply the nomogram model in the evaluation of complications in patients with HAPC and DVT who underwent anticoagulation therapy.METHODS A total of 123 patients with HAPC complicated by lower-extremity DVT were followed up for 6-12 months and divided into recurrence and non-recurrence groups according to whether they experienced recurrence of lower-extremity DVT.Clinical data and laboratory indices were compared between the groups to determine the influencing factors of thrombosis recurrence in patients with lowerextremity DVT and HAPC.This study aimed to establish and verify the value of a nomogram model for predicting the risk of thrombus recurrence.RESULTS Logistic regression analysis showed that age,immobilization during follow-up,medication compliance,compliance with wearing elastic stockings,and peripheral blood D-dimer and fibrin degradation product levels were indepen-dent risk factors for thrombosis recurrence in patients with HAPC complicated by DVT.A Hosmer-Lemeshow goodness-of-fit test demonstrated that the nomogram model established based on the results of multivariate logistic regression analysis was effective in predicting the risk of thrombosis recurrence in patients with lowerextremity DVT complicated by HAPC(χ^(2)=0.873;P>0.05).The consistency index of the model was 0.802(95%CI:0.799-0.997),indicating its good accuracy and discrimination.CONCLUSION The column chart model for the personalized prediction of thrombotic recurrence risk has good application value in predicting thrombotic recurrence in patients with lower-limb DVT combined with HAPC after discharge.
文摘目的通过小样本外显子测序与公用数据库比对进行生物信息挖掘,探寻汉族人群高原红细胞增多症(high altitude polycythemia,HAPC)变异与发病的相关性。方法纳入汉族高原红细胞增多症男性患者4例和高原健康男性5例,采集静脉血并提取DNA进行全外显子组测序;将测序数据进行功能学富集分析(gene ontology,GO&kyoto encyclopedia of genes and genomes,KEGG)构建突变基因蛋白质互作网络(protein protein interaction,PPI),选择显著性最高的基因,筛选潜在关键HAPC相关变异位点,初步探究相关变异与HAPC发病可能的机制。结果通过全外显子测序,发现HAPC相关的突变基因216个,其中表皮生长因子(epidemal growth farctor,EGF)基因在功能学富集分析(GO&KEGG),蛋白质互作网络(PPI)中具有高度显著性,初步筛选出5个潜在关键HAPC相关变异位点,通过生物信息学预测及分析,最终筛选出EGF基因上3个可能的变异c.2124G>A(p.Met708Ile)、c.2351A>T(p.Asp784Val)以及c.2759A>T(p.Glu920Val),可能是HAPC的突变位点,与HAPC发病相关。结论本研究表明汉族EGF基因的变异与HAPC发病存在相关性。EGF基因的变异可能通过干扰RNA剪接,改变RNA二级结构,影响蛋白质结构,进而影响EGF的生成及HAPC的发生发展。
基金Supported by the Grant Funded by Competitiveness Operational Programme A1.1.4.ID:P_37_798 MYELOAL-EDIAPROT(to Găman MA),No.149/26.10.2016(MySMIS2014+:106774).
文摘Myeloproliferative neoplasms(MPNs)occur due to the abnormal proliferation of one or more terminal myeloid cell lines in peripheral blood.Subjects suffering from MPNs display a high burden of cardiovascular risk factors,and thrombotic events are often the cause of death in this population of patients.Herein,we provide a brief overview of dyslipidemia and metabolic syndrome and their epidemiology in MPNs and examine the common molecular mechanisms between dyslipidemia,metabolic syndrome,and MPNs,with a special focus on cardio-vascular risk,atherosclerosis,and thrombotic events.Furthermore,we investigate the impact of dyslipidemia and metabolic syndrome on the occurrence and survival of thrombosis in MPN patients,as well as the management of dyslipidemia in MPNs,and the impact of MPN treatment on serum lipid concentrations,particularly as side/adverse effects reported in the context of clinical trials.
