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
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 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
Background:Erythrocytosis,a rare paraneoplastic syndrome,generally occurs in patients with clear cell renal cell carcinoma and has never been reported in patients with chromophobe renal cell carcinoma.Case presentatio...Background:Erythrocytosis,a rare paraneoplastic syndrome,generally occurs in patients with clear cell renal cell carcinoma and has never been reported in patients with chromophobe renal cell carcinoma.Case presentation:We report a case of a young man suffering from a giant(22-cm) mass on his left kidney.Because of a history of polycythemia vera,the patient had been treated for the condition for 9 years.Radical nephrectomy was successfully performed,and the postoperative pathologic examination confirmed a diagnosis of chromophobe renal cell carcinoma.Unexpectedly,the symptom of erythrocytosis disappeared after the surgery.Further examination and analysis were performed,and we finally attributed his erythrocytosis to chromophobe renal cell carcinoma.Conclusions:Chromophobe renal cell carcinoma could cause erythrocytosis,but the clear-cut mechanism needs further research.Secondary erythrocytosis such as those related with renal tumors should be taken into consideration during the diagnosis of polycythemia vera.展开更多
Essential thrombocythemia(ET) and polycythemia vera(PV) frequently present with erythromelalgia and acrocyanotic complications, migraine-like microvascular cerebral and ocular transient ischemic attacks(MIAs) and/or a...Essential thrombocythemia(ET) and polycythemia vera(PV) frequently present with erythromelalgia and acrocyanotic complications, migraine-like microvascular cerebral and ocular transient ischemic attacks(MIAs) and/or acute coronary disease. The spectrum of MIAs in ET range from poorly localized symptoms of transient unsteadiness, dysarthria and scintillating scotoma to focal symptoms of transient monocular blindness, transient mono- or hemiparesis or both. The attacks all have a sudden onset, occur sequentially rather than simultaneously, last for a few seconds to several minutes and are usually associated with a dull, pulsatile or migraine-like headache. Increased hematocrit and blood viscosity in PV patients aggravate the microvascular ischemic syndrome of thrombocythemia to major arterial and venous thrombotic complications. Phlebotomy to correct hematocrit to normal in PV significantly reduces major arterial and venous thrombotic complications, but fails to prevent the platelet-mediated erythromelalgia and MIAs. Complete long-term relief of the erythromelalgic microvascular disturbances, MIAs and major thrombosis in ET and PV patients can be obtained with low dose aspirin and platelet reduction to normal, but not with anticoagulation. Skin punch biopsies from the erythromelalgic area show fibromuscular intimal proliferation of arterioles complicated by occlusive plateletrich thrombi leading to acrocyanotic ischemia. Symptomatic ET patients with erythromelalgic microvascular disturbances have shortened platelet survival, increased platelet activation markers β-thromboglobulin(β-TG), platelet factor 4(PF4) and thrombomoduline(TM), increased urinary thromboxane B2(TXB2) excretion, and no activation of the coagulation markers thrombin fragments F1+2 and fibrin degradation products. Inhibition of platelet cyclooxygenase(COX1) by aspirin is followed by the disappearance and no recurrence of microvascular disturbances, increase in platelet number, correction of the shortened platelet survival times to normal, and reduction of increased plasma levels of β-TG, PF4, TM and urinary TXB2 excretion to normal. These results indicate that platelet-mediated fibromuscular intimal proliferation and platelet-rich thrombi in the peripheral, cerebral and coronary end-arterial microvasculature are responsible for the erythromelalgic ischemic complica-tions, MIAs and splanchnic vein thrombosis. Baseline platelet P-selectin levels and arachidonic acid induced COX1 mediated platelet activation showed a highly significant increase of platelet P-selectin expression(not seen in ADP and collagen stimulated platelets), which was significantly higher in JAK2V617 F mutated compared to JAK2 wild type ET.展开更多
Pulmonary vein thrombosis(PVT) is a rarely encountered disease entity with varied clinical presentations. It is usually associated with lung carcinoma, lung surgeries and as a complication of the radiofrequency cathet...Pulmonary vein thrombosis(PVT) is a rarely encountered disease entity with varied clinical presentations. It is usually associated with lung carcinoma, lung surgeries and as a complication of the radiofrequency catheter ablation procedure for atrial fibrillation. Its clinical manifestations can vary from mild hemoptysis to lung infarction with hemodynamic compromise. A 76-year-old male presented with a 2-d history of pleuritic left sided chest pain. His past medical history included polycythemia vera, atrial fibrillation, coronary artery disease, pulmonary embolism and pulmonary hypertension. Chest radiograph was normal, troponins were normal and the 12-lead electrocardiogram did not show any ischemic changes. A computerized tomography pulmonary angiogram revealed a filling defect in the left lower lobe pulmonary vein. He was treated with subcutaneous enoxaparin and his symptoms improved. This case highlights a rare etiology of chest pain and the first reported case of the association of polycythemia vera and pulmonary vein thrombosis. A high index of suspicion is required for appropriate diagnostic work up. PVT can mimic pulmonary embolism. The diagnostic work up and treatment strategies depend on acuity of presentation.展开更多
Polycythemia vera has been reported as a known condition in cats as early as 1966. This condition manifests as an increased mass in red blood cells and elevated hematocrit and is defined as an idiopathic chronic myelo...Polycythemia vera has been reported as a known condition in cats as early as 1966. This condition manifests as an increased mass in red blood cells and elevated hematocrit and is defined as an idiopathic chronic myeloproliferative disorder. The patient described in this paper presented with hyperemic gums and pinna and an acute onset of progressive ataxia and lethargy. Several possible underlying primary conditions such as cardiac disease and renal malignancy were excluded by running basic blood work and radiographic imaging. Initial blood work revealed a significantly elevated packed cell volume (88%). After diagnosis, treatment with phlebotomy and chemotherapy lead to a reduction in hematocrit and elimination of neurologic signs. This case study represents the diagnosis and successful management of this disease in a private practice setting. Polycythemia vera is relatively uncommon in dogs and cats, but should still be considered in cases of neurologic disorders, especially with the presence of bright red ears, paws, or gums.展开更多
Carcinoid tumors have been reported in a wide range of organs but most frequently involve the gastrointestinal tract; however, duodenal carcinoid tumors are rare. We report a 50-year-old male patient complaining of mu...Carcinoid tumors have been reported in a wide range of organs but most frequently involve the gastrointestinal tract; however, duodenal carcinoid tumors are rare. We report a 50-year-old male patient complaining of multiple melenas for 3 wk. The panendoscopy and endoscopic retrograde cholangiopancreaticography revealed swelling accessory papilla with an ulcer. The biopsy taken showed a carcinoid tumor. The lesion was removed by wide resection. Patient was found to have an abnormal blood cell count during the follow-up period with elevated levels of hemoglobin and hematocrit of 21.2 g/dL and 63.5%,respectively, thrombocytosis of 501 000/μL, and leukocytosis of 20 410/μL. He was diagnosed as a polycythemia vera by a hematologist after further evaluation. He received periodic phlebotomy and hydroxyurea treatment. The response was good and his hematocrit was stabilized by periodic phlebotomy in the range of 44-49% during the last 2 years. The possible origin of UGI bleeding by a duodenal carcinoid tumor, although rare, should be considered. There has been one case report of a duodenal carcinoid tumor that involved accessory papilla of the pancreas divisum and one case report of metastatic carcinoid tumor associated with polycythemia vera. It is different in our patient as compared with the latter report, which mentioned a polycythemia vera patient who was found to have a metastatic carcinoid in the 17 years follow-up period. Chemotherapy had been given before the carcinoid tumor was revealed. Our patient had no previous chemotherapy for polycythemia vera before he was found to have duodenal carcinoid tumor; this excludes the possibility of chemotherapy induced carcinoid tumor, although it had been suspected in the previous report. In our patient, the existence of both diseases may be by predisposition of each other since both diseases have an increased incidence of other neoplasm, or they may be coexistent incidentally.展开更多
BACKGROUND Acute arterial embolism of the extremities is a surgical emergency. Atrialfibrillation is the major etiology of acute arterial embolism of the extremities.Emergency femoral artery thrombectomy can successfu...BACKGROUND Acute arterial embolism of the extremities is a surgical emergency. Atrialfibrillation is the major etiology of acute arterial embolism of the extremities.Emergency femoral artery thrombectomy can successfully treat this issue.However, polycythemia vera (PV) may sometimes explain this medicalemergency. Recurrent thrombosis in the lower extremities after thrombectomycan be found in patients with PV, and reoperation is needed for this condition.CASE SUMMARY A 68-year-old man in China suffered from sudden pain in the left lower extremityfor 14 h. The examination in the emergency department showed a diagnosis ofacute arterial embolism of the extremities combined with PV. The patient’scomplaint disappeared after repeat emergency thrombectomy.CONCLUSION Patients with acute arterial embolism of the extremities should be treatedcarefully, especially those who have recurrent thrombosis after emergencythrombectomy. Clinicians should be aware of PV, a rare cause of acute arterialembolism of the extremities. The combination of thrombectomy, phlebotomy, andantiplatelet and anticoagulant drugs may be a suitable therapeutic regimen forthese patients.展开更多
BACKGROUNDPolycythemia vera (PV) is a chronic myeloproliferative disorder characterized byan increase in red blood cells in the peripheral blood. Previous work has reportedthe occurrence of thrombosis or hemorrhage ar...BACKGROUNDPolycythemia vera (PV) is a chronic myeloproliferative disorder characterized byan increase in red blood cells in the peripheral blood. Previous work has reportedthe occurrence of thrombosis or hemorrhage arising in the cerebral vasculaturesecondary to PV. However, hemorrhagic transformation after PV-associated acuteischemic stroke has not been previously described.CASE SUMMARYWe herein present two cases of PV where hemorrhagic transformation occurredafter an acute ischemic stroke. Case 1 was a 57-year-old woman with a history ofhypertension who was admitted for left-sided weakness. Case 2 was a 68-year-oldman who was admitted for a 10-d sudden left arm weakness. Imaging examinationsfor the two patients revealed hemorrhagic transformation after acuteischemic stroke. Both patients had JAK-2-V617F mutation and receivedantiplatelet therapy. Both of them had a good prognosis during the follow-up.CONCLUSIONThis report suggested that hemorrhagic transformation may occur in acuteischemic stroke caused by PV. Antiplatelet drugs do not seem to influence thelong-term outcomes in such patients. Future research should focus on establishinga standard antiplatelet treatment strategy for this condition.展开更多
Background: Polycythemia vera is a possible cause of recurrent ischemic stroke which can be prevented. Aim: Describe a junctional ischemic stroke without large arterial trunks stenosis associated with an acute coronar...Background: Polycythemia vera is a possible cause of recurrent ischemic stroke which can be prevented. Aim: Describe a junctional ischemic stroke without large arterial trunks stenosis associated with an acute coronary syndrome. Case Presentation: A 66-years-old man was admitted for abrupt recurrent right hemiparesis related to bilateral and junctional ischemic stroke lesions. He had a medical history of a vertebrobasilar ischemic stroke concurrent with an acute coronary syndrome with normal coronary arteries. Transthoracic echocardiogram showed small apical akinesia. Hemoglobin level was 18.9 g/dl with a hematocrit of 57.6%. The endogenous erythropoietin was 1.3 mIU/ml with JAK2 V617F mutation positivity (37%). After eight months of treatment (hydroxycarbamide + aspirin + allopurinol) hemoglobin was 12.5 g/dL. Conclusion: This case illustrates the most suggestive features of PV particularly the ischemic stroke junctional topography.展开更多
BACKGROUND Polycythemia vera(PV),often attributed to the JAK2 V617F mutation,is characterized by enhanced red blood cell counts in the peripheral blood.PV-associated renal disease is clinically rare;to date,there have...BACKGROUND Polycythemia vera(PV),often attributed to the JAK2 V617F mutation,is characterized by enhanced red blood cell counts in the peripheral blood.PV-associated renal disease is clinically rare;to date,there have been reports of other chronic kidney diseases related to PV,but no reports on PV-associated minimal change disease.CASE SUMMARY A 37-year-old man presented with proteinuria and high red blood cell count on January 4,2021.The patient underwent bone marrow and renal biopsies,then was subsequently diagnosed with PV and minimal change in disease.Hydroxyurea was administered and proteinuria remission was achieved.The patient’s last visit was on April 14,2022.CONCLUSION We inferred that there may be a causal relationship between PV and minimal change disease.展开更多
Despite its association with vascular events such as myocardial and cerebral infarction, polycythemia vera (PV) is characterized by low serum total cholesterol levels. Because several sex hormones are derived from cho...Despite its association with vascular events such as myocardial and cerebral infarction, polycythemia vera (PV) is characterized by low serum total cholesterol levels. Because several sex hormones are derived from cholesterol, statins may induce hypogodanism in male patients. Therefore, we assessed the relationship between serum total cholesterol and sex hormone levels according to gender. Medical records of 41 patients with erythrocytosis (hemoglobin concentrations: men >18.5 g/dL;women >16.5 g/dL) collected between August 2005 and December 2014 were reviewed for patient age, and gender, as well as clinical hematology, biochemistry, and endocrinology laboratory findings. Serum levels of testosterone were lower in men with PV than in patients with reactive erythrocytosis (RE) (PV: 385 ± 78 ng/mL versus RE: 529 ± 46 ng/mL). However, serum levels of testosterone in women with PV were comparable to those in patients in the nonerythrocytic group (PV: 20.5 ± 3.5 ng/mL versus non-erythrocytic group: 21.0 ± 4.3 ng/mL). Serum levels of testosterone were not related to serum levels of cholesterol. Therefore, we speculated that lower testosterone levels were not due to lower serum levels of cholesterol, a known adverse effect of statin. In conclusion, we report for the first time that serum levels of testosterone were lower in male PV patients than in those with RE;however, serum levels of testosterone in female patients with PV were not lower.展开更多
Aloe vera plant is known worldwide for its medicinal properties and application in gel-based products such as shampoo,soap,and sunscreen.However,the demand for these gel-based products has led to a surplus production ...Aloe vera plant is known worldwide for its medicinal properties and application in gel-based products such as shampoo,soap,and sunscreen.However,the demand for these gel-based products has led to a surplus production of Aloe vera processing waste.An Aloe vera gel processing facility could generate up to 4000 kg of Aloe vera waste per month.Currently the Aloe vera waste is being disposed to the landfill or used as fertilizer.A sustainable management system for the Aloe vera processing waste should be considered,due to the negative societal and environmental impacts of the currents waste disposal methods.Therefore,this review focuses on various approaches that can be used to valorize Aloe vera waste into value-added products,such as animal and aquaculture feeds,biosorbents,biofuel and natural polymers.Researchers have reported Aloe vera waste for environmental applications biosorbents used for wastewater treatment of various pollutants.Several studies have also reported on the valorization of Aloe vera waste for production of biofuels such as bioethanol,mixed alcohol fuels,biogas and syngas.Aloe vera waste could also be valorized through isolation and synthesis of natural polymers for application in wound dressing,tissue engineering and drug delivery systems.Aloe vera waste valorization was also reviewed through extraction of value-added bioactive compounds such as aloe-emodin,aloin and aloeresin.These value-added bioactive compounds have various applications in the cosmetics(non-steroidal anti-inflammatory,tyrosinase inhibitors)and pharmaceutical(anticancer agent and COVID 19 inhibitors)industry.展开更多
This review summarizes the main chemical composition of Aloe vera flowers and introduces their antioxidant,anti-aging,skin whitening,skin moisturizing,anti-skin irritation,and anti-skin allergy effects.Suggestions for...This review summarizes the main chemical composition of Aloe vera flowers and introduces their antioxidant,anti-aging,skin whitening,skin moisturizing,anti-skin irritation,and anti-skin allergy effects.Suggestions for the application of Aloe v.flowers in cosmetics are provided.展开更多
The search for functional foods with health benefits has increased,and yogurt represents one of these products due to their properties,such as,source of probiotics that favour the gastrointestinal system,contains vita...The search for functional foods with health benefits has increased,and yogurt represents one of these products due to their properties,such as,source of probiotics that favour the gastrointestinal system,contains vitamins,minerals and improves the action of enzymes.Present work investigated bromatological,microbiological,sensorial properties and shelf-life of different yogurt goat milk formulations with Aloe vera and natural sweeteners.The goat yogurt with 99.40%A.vera pulp and 0.60%S.rebaudiana presented the best nutritional composition,being suitable for human consumption representing an innovative product with a great functional potential.The synergy between the ingredients used in the yogurt processing resulted in an innovative product to the market.Goat milk has a lower cost per liter in comparison with cow milk,Aloe vera is accessible due to its fast-vegetative propagation,Stevia Rebaudiana has a great popularity for being a natural sweetener with no calories,and all these products are produced in México.展开更多
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.
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 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.
基金financially supported by National Natural Science Foundation of China(Nos.81372335,81402108)the China Postdoctoral Science Foundation(No.2014M551915)
文摘Background:Erythrocytosis,a rare paraneoplastic syndrome,generally occurs in patients with clear cell renal cell carcinoma and has never been reported in patients with chromophobe renal cell carcinoma.Case presentation:We report a case of a young man suffering from a giant(22-cm) mass on his left kidney.Because of a history of polycythemia vera,the patient had been treated for the condition for 9 years.Radical nephrectomy was successfully performed,and the postoperative pathologic examination confirmed a diagnosis of chromophobe renal cell carcinoma.Unexpectedly,the symptom of erythrocytosis disappeared after the surgery.Further examination and analysis were performed,and we finally attributed his erythrocytosis to chromophobe renal cell carcinoma.Conclusions:Chromophobe renal cell carcinoma could cause erythrocytosis,but the clear-cut mechanism needs further research.Secondary erythrocytosis such as those related with renal tumors should be taken into consideration during the diagnosis of polycythemia vera.
文摘Essential thrombocythemia(ET) and polycythemia vera(PV) frequently present with erythromelalgia and acrocyanotic complications, migraine-like microvascular cerebral and ocular transient ischemic attacks(MIAs) and/or acute coronary disease. The spectrum of MIAs in ET range from poorly localized symptoms of transient unsteadiness, dysarthria and scintillating scotoma to focal symptoms of transient monocular blindness, transient mono- or hemiparesis or both. The attacks all have a sudden onset, occur sequentially rather than simultaneously, last for a few seconds to several minutes and are usually associated with a dull, pulsatile or migraine-like headache. Increased hematocrit and blood viscosity in PV patients aggravate the microvascular ischemic syndrome of thrombocythemia to major arterial and venous thrombotic complications. Phlebotomy to correct hematocrit to normal in PV significantly reduces major arterial and venous thrombotic complications, but fails to prevent the platelet-mediated erythromelalgia and MIAs. Complete long-term relief of the erythromelalgic microvascular disturbances, MIAs and major thrombosis in ET and PV patients can be obtained with low dose aspirin and platelet reduction to normal, but not with anticoagulation. Skin punch biopsies from the erythromelalgic area show fibromuscular intimal proliferation of arterioles complicated by occlusive plateletrich thrombi leading to acrocyanotic ischemia. Symptomatic ET patients with erythromelalgic microvascular disturbances have shortened platelet survival, increased platelet activation markers β-thromboglobulin(β-TG), platelet factor 4(PF4) and thrombomoduline(TM), increased urinary thromboxane B2(TXB2) excretion, and no activation of the coagulation markers thrombin fragments F1+2 and fibrin degradation products. Inhibition of platelet cyclooxygenase(COX1) by aspirin is followed by the disappearance and no recurrence of microvascular disturbances, increase in platelet number, correction of the shortened platelet survival times to normal, and reduction of increased plasma levels of β-TG, PF4, TM and urinary TXB2 excretion to normal. These results indicate that platelet-mediated fibromuscular intimal proliferation and platelet-rich thrombi in the peripheral, cerebral and coronary end-arterial microvasculature are responsible for the erythromelalgic ischemic complica-tions, MIAs and splanchnic vein thrombosis. Baseline platelet P-selectin levels and arachidonic acid induced COX1 mediated platelet activation showed a highly significant increase of platelet P-selectin expression(not seen in ADP and collagen stimulated platelets), which was significantly higher in JAK2V617 F mutated compared to JAK2 wild type ET.
文摘Pulmonary vein thrombosis(PVT) is a rarely encountered disease entity with varied clinical presentations. It is usually associated with lung carcinoma, lung surgeries and as a complication of the radiofrequency catheter ablation procedure for atrial fibrillation. Its clinical manifestations can vary from mild hemoptysis to lung infarction with hemodynamic compromise. A 76-year-old male presented with a 2-d history of pleuritic left sided chest pain. His past medical history included polycythemia vera, atrial fibrillation, coronary artery disease, pulmonary embolism and pulmonary hypertension. Chest radiograph was normal, troponins were normal and the 12-lead electrocardiogram did not show any ischemic changes. A computerized tomography pulmonary angiogram revealed a filling defect in the left lower lobe pulmonary vein. He was treated with subcutaneous enoxaparin and his symptoms improved. This case highlights a rare etiology of chest pain and the first reported case of the association of polycythemia vera and pulmonary vein thrombosis. A high index of suspicion is required for appropriate diagnostic work up. PVT can mimic pulmonary embolism. The diagnostic work up and treatment strategies depend on acuity of presentation.
文摘Polycythemia vera has been reported as a known condition in cats as early as 1966. This condition manifests as an increased mass in red blood cells and elevated hematocrit and is defined as an idiopathic chronic myeloproliferative disorder. The patient described in this paper presented with hyperemic gums and pinna and an acute onset of progressive ataxia and lethargy. Several possible underlying primary conditions such as cardiac disease and renal malignancy were excluded by running basic blood work and radiographic imaging. Initial blood work revealed a significantly elevated packed cell volume (88%). After diagnosis, treatment with phlebotomy and chemotherapy lead to a reduction in hematocrit and elimination of neurologic signs. This case study represents the diagnosis and successful management of this disease in a private practice setting. Polycythemia vera is relatively uncommon in dogs and cats, but should still be considered in cases of neurologic disorders, especially with the presence of bright red ears, paws, or gums.
文摘Carcinoid tumors have been reported in a wide range of organs but most frequently involve the gastrointestinal tract; however, duodenal carcinoid tumors are rare. We report a 50-year-old male patient complaining of multiple melenas for 3 wk. The panendoscopy and endoscopic retrograde cholangiopancreaticography revealed swelling accessory papilla with an ulcer. The biopsy taken showed a carcinoid tumor. The lesion was removed by wide resection. Patient was found to have an abnormal blood cell count during the follow-up period with elevated levels of hemoglobin and hematocrit of 21.2 g/dL and 63.5%,respectively, thrombocytosis of 501 000/μL, and leukocytosis of 20 410/μL. He was diagnosed as a polycythemia vera by a hematologist after further evaluation. He received periodic phlebotomy and hydroxyurea treatment. The response was good and his hematocrit was stabilized by periodic phlebotomy in the range of 44-49% during the last 2 years. The possible origin of UGI bleeding by a duodenal carcinoid tumor, although rare, should be considered. There has been one case report of a duodenal carcinoid tumor that involved accessory papilla of the pancreas divisum and one case report of metastatic carcinoid tumor associated with polycythemia vera. It is different in our patient as compared with the latter report, which mentioned a polycythemia vera patient who was found to have a metastatic carcinoid in the 17 years follow-up period. Chemotherapy had been given before the carcinoid tumor was revealed. Our patient had no previous chemotherapy for polycythemia vera before he was found to have duodenal carcinoid tumor; this excludes the possibility of chemotherapy induced carcinoid tumor, although it had been suspected in the previous report. In our patient, the existence of both diseases may be by predisposition of each other since both diseases have an increased incidence of other neoplasm, or they may be coexistent incidentally.
文摘BACKGROUND Acute arterial embolism of the extremities is a surgical emergency. Atrialfibrillation is the major etiology of acute arterial embolism of the extremities.Emergency femoral artery thrombectomy can successfully treat this issue.However, polycythemia vera (PV) may sometimes explain this medicalemergency. Recurrent thrombosis in the lower extremities after thrombectomycan be found in patients with PV, and reoperation is needed for this condition.CASE SUMMARY A 68-year-old man in China suffered from sudden pain in the left lower extremityfor 14 h. The examination in the emergency department showed a diagnosis ofacute arterial embolism of the extremities combined with PV. The patient’scomplaint disappeared after repeat emergency thrombectomy.CONCLUSION Patients with acute arterial embolism of the extremities should be treatedcarefully, especially those who have recurrent thrombosis after emergencythrombectomy. Clinicians should be aware of PV, a rare cause of acute arterialembolism of the extremities. The combination of thrombectomy, phlebotomy, andantiplatelet and anticoagulant drugs may be a suitable therapeutic regimen forthese patients.
文摘BACKGROUNDPolycythemia vera (PV) is a chronic myeloproliferative disorder characterized byan increase in red blood cells in the peripheral blood. Previous work has reportedthe occurrence of thrombosis or hemorrhage arising in the cerebral vasculaturesecondary to PV. However, hemorrhagic transformation after PV-associated acuteischemic stroke has not been previously described.CASE SUMMARYWe herein present two cases of PV where hemorrhagic transformation occurredafter an acute ischemic stroke. Case 1 was a 57-year-old woman with a history ofhypertension who was admitted for left-sided weakness. Case 2 was a 68-year-oldman who was admitted for a 10-d sudden left arm weakness. Imaging examinationsfor the two patients revealed hemorrhagic transformation after acuteischemic stroke. Both patients had JAK-2-V617F mutation and receivedantiplatelet therapy. Both of them had a good prognosis during the follow-up.CONCLUSIONThis report suggested that hemorrhagic transformation may occur in acuteischemic stroke caused by PV. Antiplatelet drugs do not seem to influence thelong-term outcomes in such patients. Future research should focus on establishinga standard antiplatelet treatment strategy for this condition.
文摘Background: Polycythemia vera is a possible cause of recurrent ischemic stroke which can be prevented. Aim: Describe a junctional ischemic stroke without large arterial trunks stenosis associated with an acute coronary syndrome. Case Presentation: A 66-years-old man was admitted for abrupt recurrent right hemiparesis related to bilateral and junctional ischemic stroke lesions. He had a medical history of a vertebrobasilar ischemic stroke concurrent with an acute coronary syndrome with normal coronary arteries. Transthoracic echocardiogram showed small apical akinesia. Hemoglobin level was 18.9 g/dl with a hematocrit of 57.6%. The endogenous erythropoietin was 1.3 mIU/ml with JAK2 V617F mutation positivity (37%). After eight months of treatment (hydroxycarbamide + aspirin + allopurinol) hemoglobin was 12.5 g/dL. Conclusion: This case illustrates the most suggestive features of PV particularly the ischemic stroke junctional topography.
基金Supported by the National Natural Science Foundation of China,No.81874437 and 81904126Science and Technology Commission of Shanghai Municipality,No.20Y21901800.
文摘BACKGROUND Polycythemia vera(PV),often attributed to the JAK2 V617F mutation,is characterized by enhanced red blood cell counts in the peripheral blood.PV-associated renal disease is clinically rare;to date,there have been reports of other chronic kidney diseases related to PV,but no reports on PV-associated minimal change disease.CASE SUMMARY A 37-year-old man presented with proteinuria and high red blood cell count on January 4,2021.The patient underwent bone marrow and renal biopsies,then was subsequently diagnosed with PV and minimal change in disease.Hydroxyurea was administered and proteinuria remission was achieved.The patient’s last visit was on April 14,2022.CONCLUSION We inferred that there may be a causal relationship between PV and minimal change disease.
文摘Despite its association with vascular events such as myocardial and cerebral infarction, polycythemia vera (PV) is characterized by low serum total cholesterol levels. Because several sex hormones are derived from cholesterol, statins may induce hypogodanism in male patients. Therefore, we assessed the relationship between serum total cholesterol and sex hormone levels according to gender. Medical records of 41 patients with erythrocytosis (hemoglobin concentrations: men >18.5 g/dL;women >16.5 g/dL) collected between August 2005 and December 2014 were reviewed for patient age, and gender, as well as clinical hematology, biochemistry, and endocrinology laboratory findings. Serum levels of testosterone were lower in men with PV than in patients with reactive erythrocytosis (RE) (PV: 385 ± 78 ng/mL versus RE: 529 ± 46 ng/mL). However, serum levels of testosterone in women with PV were comparable to those in patients in the nonerythrocytic group (PV: 20.5 ± 3.5 ng/mL versus non-erythrocytic group: 21.0 ± 4.3 ng/mL). Serum levels of testosterone were not related to serum levels of cholesterol. Therefore, we speculated that lower testosterone levels were not due to lower serum levels of cholesterol, a known adverse effect of statin. In conclusion, we report for the first time that serum levels of testosterone were lower in male PV patients than in those with RE;however, serum levels of testosterone in female patients with PV were not lower.
文摘Aloe vera plant is known worldwide for its medicinal properties and application in gel-based products such as shampoo,soap,and sunscreen.However,the demand for these gel-based products has led to a surplus production of Aloe vera processing waste.An Aloe vera gel processing facility could generate up to 4000 kg of Aloe vera waste per month.Currently the Aloe vera waste is being disposed to the landfill or used as fertilizer.A sustainable management system for the Aloe vera processing waste should be considered,due to the negative societal and environmental impacts of the currents waste disposal methods.Therefore,this review focuses on various approaches that can be used to valorize Aloe vera waste into value-added products,such as animal and aquaculture feeds,biosorbents,biofuel and natural polymers.Researchers have reported Aloe vera waste for environmental applications biosorbents used for wastewater treatment of various pollutants.Several studies have also reported on the valorization of Aloe vera waste for production of biofuels such as bioethanol,mixed alcohol fuels,biogas and syngas.Aloe vera waste could also be valorized through isolation and synthesis of natural polymers for application in wound dressing,tissue engineering and drug delivery systems.Aloe vera waste valorization was also reviewed through extraction of value-added bioactive compounds such as aloe-emodin,aloin and aloeresin.These value-added bioactive compounds have various applications in the cosmetics(non-steroidal anti-inflammatory,tyrosinase inhibitors)and pharmaceutical(anticancer agent and COVID 19 inhibitors)industry.
文摘This review summarizes the main chemical composition of Aloe vera flowers and introduces their antioxidant,anti-aging,skin whitening,skin moisturizing,anti-skin irritation,and anti-skin allergy effects.Suggestions for the application of Aloe v.flowers in cosmetics are provided.
文摘The search for functional foods with health benefits has increased,and yogurt represents one of these products due to their properties,such as,source of probiotics that favour the gastrointestinal system,contains vitamins,minerals and improves the action of enzymes.Present work investigated bromatological,microbiological,sensorial properties and shelf-life of different yogurt goat milk formulations with Aloe vera and natural sweeteners.The goat yogurt with 99.40%A.vera pulp and 0.60%S.rebaudiana presented the best nutritional composition,being suitable for human consumption representing an innovative product with a great functional potential.The synergy between the ingredients used in the yogurt processing resulted in an innovative product to the market.Goat milk has a lower cost per liter in comparison with cow milk,Aloe vera is accessible due to its fast-vegetative propagation,Stevia Rebaudiana has a great popularity for being a natural sweetener with no calories,and all these products are produced in México.
