Cardiac hypertrophy in polycythemia vera:A case report and review of literature

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.

Evaluation of Hemoglobin and Serum Erythropoietin Levels in Patients with Polycythemia Vera and Secondary Polycythemia

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. .

Does Ruxolitinib, in Comparison to Best Available Therapy (BAT), Improve Pruritis Symptoms in Patients with Polycythemia Vera?

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.

Carcinoid tumor of the duodenum and accessory papilla associated with polycythemia vera

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.

Hemorrhagic transformation after acute ischemic stroke caused by polycythemia vera:Report of two case

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.

Minimal change disease caused by polycythemia vera: A case report

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.

Recurrent thrombosis in the lower extremities after thrombectomy in a patient with polycythemia vera:A case report

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.

Recurrent Ischemic Stroke Revealing Polycythemia Vera

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.

Serum Levels of Testosterone in Patients with Polycythemia Vera

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.

Erythrocytosis caused by giant chromophobe renal cell carcinoma:a case report indicating a 9.year misdiagnosis of polycythemia vera

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.

Pulmonary vein thrombosis in a patient with polycythemia vera

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.

Aspirin responsive platelet thrombophilia in essential thrombocythemia and polycythemia vera

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.

Case Report on Feline Polycythemia Vera

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.

Endogenous erythroid colony assay in patients with polycythemia vera and its clinical significance

Background Polycythemia vera (PV) is a malignant disorder of hemaopoietic stem cells which is characterized by clonal hyperproliferation and a low rate of apoptosis. This study was to assess endogenous erythroid colony (EEC) formation in the bone marrow of PV patients and determine its clinical significance Methods The bone marrow mononuclear cells of 26 patients with PV, 2 patients with secondary erythrocytosis (SE), and 19 normal controls were cultured by Marsh's method for EEC evaluation, and the clinical significance was evaluated Results EECs appeared in 25 patients with PV but not in 2 patients with SE and 19 normal controls The number of EECs and the EEC ratio [EEC/erythropoietin (EPO)-dependent colony forming unit-erythroid (CFU-E)] in PV patients positively correlated with hemoglobin (Hb) levels Their EEC number did not correlate with white blood cell (WBC) counts, platelet (PLT) counts, or leukocyte alkaline phosphatase (LAP) scores Their EEC did not correlate with serum EPO levels Fifteen patients with PV were treated with hydroxyurea (Hu) and/or interferon-alpha (IFN-α) Their EEC ratio before treatment positively correlated with the treatment time required for complete remission (CR) and negatively correlated with the time before relapse The EEC numbers of 7 PV patients treated with Hu/IFN-α decreased after the blood cell counts dropped to normal levels There was a positive correlation between the EEC ratio and the incidence of attacks of vascular thrombosis in PV patients The numbers of apoptosised bone marrow mononuclear cells in PV patients were lower than those in normal controls The EEC numbers of PV patients negatively correlated with the rate of apoptosis of bone marrow mononuclear cells Conclusions EEC formation is characteristic in PV patients EEC number in PV patients positively correlates with Hb levels, the time required for CR, and the incidence of attacks of vascular thrombosis EEC number negatively correlates with the time before relapse Bone marrow suppressive treatment might decrease EEC number Thus, EEC number is a sensitive and specific parameter reflecting the abnormal hematopoietic clone burden induced by polycythemia vera EEC number is an important diagnostic parameter for PV patients

Decreased serum prohepcidin concentration in patients with polycythemia vera

Objective： Iron deficiency is a common complication in patients with polycythemia vera （PV）. Hepcidin is a principal regulator of iron homeostasis. The aim of our study was to assess prohepcidin, a hepcidin precursor, and other iron status pa- rameters in the serum of PV patients. Methods： The study was performed in 60 patients （F/M 26/34） aged 38-84 （66＋ 10） years. The control group consisted of 20 healthy volunteers, age and sex matched. The following parameters were determined in blood serum samples： prohepcidin concentration, iron content, unsaturated iron binding capacity （UIBC）, total iron binding capacity （TIBC）, transferrin saturation （TfS）, and concentrations of ferritin and soluble transferrin receptor （sTfR）. Results： All PV patients showed significantly lower levels of prohepcidin, higher levels of sTIR and TIBC compared to the control group. 40% of the patients from the study group showed concentrations of ferritin below the normal range and significantly lower levels of serum iron and TfS, and significantly higher levels of sTIR, UIBC and TIBC in comparison with the rest of the study group. In this group of patients, prohepcidin concentrations were significantly lower than those in other patients. Conclusion： The results indicate that PV patients suffer from iron metabolism disorders. The decreased serum level ofprohepcidin in PV patients may be a result of iron deficiency.

Observation of a higher JAK2 V617F homozygous mutated clone in polycythemia vera compared to essential thrombocythemia

Single-tube bi-directional allele specific amp-lification(SB-ASA)and real-time quantitative polymer-ase chain reaction(RQ-PCR)assays were developed and performed for JAK2V617F detection on 40 polycythemia vera(PV)samples,31 essential thrombocythemia(ET)samples,40 acute leukemia samples,and 40 healthy con-trol samples.Differences between detect limitations of the two assays and their influence on the mutation detection rate were analyzed.The results showed that in some sam-ples,the JAK2V617F burden was as low as nearly 1%,and thus more JAK2V617F-positive samples were detected by RQ-PCR than by SB-ASA assay due to the former higher detect limitation.Mutation allele ratios in PV and ET samples and their relevance to biological char-acteristics were also analyzed.The results showed that the mutation allele ratio was 0.436±0.261 in PV,higher than the 0.216±0.207 in ET;percentage of certainly homo-zygous mutation carriers in PV was 40.54%,higher than the 10%in ET.However,statistical analysis showed no relevance between mutation allele burden and sex or age.Our result shows that the pathogenesis of PV and ET may be related to the mutation allele burden of JAK2V617F.

血小板参数对骨髓增殖性肿瘤患者动脉并发症的预测价值

目的:通过分析真性红细胞增多症(PV)及原发性血小板增多症(ET)患者动脉事件发生情况和血小板参数水平,探讨骨髓增殖性肿瘤(MPN)患者的血小板参数特点及其与动脉并发症的关系。方法:回顾性分析2017年8月至2022年8月于首都医科大学附属北京安贞医院血液内科初诊的PV、ET患者的临床和实验室资料。结果:入选86例MPN患者,其中PV 44例、ET 42例,男性46例、女性40例,中位发病年龄61(23-83)岁。PV患者的JAK2V617F基因突变率、骨髓巨核细胞数量、脾肿大的发生率,以及WBC、HGB、HCT、PDW、MPV、P-LCR水平均明显高于ET患者(P<0.05),PLT和PCT水平显著低于ET患者(P<0.01)。22例(50%)PV患者合并动脉事件,其中≥2个部位发生动脉狭窄者12例,动脉事件中,合并缺血性脑卒中的PV患者PDW高于无缺血性脑卒中的PV患者(P=0.003),≥2个部位发生动脉狭窄的PV患者PDW高于≤1个部位动脉狭窄的PV患者(P=0.037)。23例(54.8%)ET患者合并动脉事件,≥2个部位发生动脉狭窄者7例,动脉事件中,合并缺血性脑卒中的ET患者PCT高于无缺血性脑卒中的ET患者(P=0.037),≥2个部位发生动脉狭窄的ET患者PCT高于≤1个部位动脉狭窄的ET患者(P=0.049)。二元logistic回归分析结果显示,PDW、PCT升高分别是PV、ET患者发生缺血性脑卒中的危险因素(P<0.05)。结论:PV和ET患者的血小板参数具有明显不同的特点,PDW和PCT升高分别预示着PV和ET患者发生缺血性脑卒中的风险较高。

骨髓增殖性肿瘤患者高血压患病的临床特征分析

目的探索骨髓增殖性肿瘤(MPN)患者高血压患病的临床特征。方法回顾性收集2010年5月至2020年5月首都医科大学附属北京安贞医院收治住院的103例MPN患者[包括真性红细胞增多症(PV)患者(PV组)61例和原发性血小板增多症(ET)患者(ET组)42例]的临床资料。统计PV组和ET组患者高血压患病率和年龄分布情况,比较2组中高血压患者的临床特征差异。结果本研究中MPN患者总体的高血压患病率为52.4%(54/103),以55~64岁、75~84岁组高血压患病相对比例较高,分别为17例/27例、5例/7例;而25~44岁组高血压患者均为MPN继发性高血压。PV组高血压患者年龄明显小于、血小板计数水平低于ET组,男性比例、JAK2V617F基因突变率、男性和女性血肌酐水平、血红蛋白、红细胞压积均高于ET组(均P<0.05)。相关性分析显示PV组中高血压患者的收缩压水平与白细胞计数呈正相关性,2组中高血压患者的左心室射血分数均与白细胞计数呈负相关性(r=0.453,P=0.047;r=-0.448,P=0.019;r=-0.491,P=0.049)。PV组继发性高血压患者MPN病史长于,舒张压水平、男性和女性血肌酐水平、JAK2V617F基因突变率、血红蛋白和红细胞压积水平均明显高于ET组,血小板计数水平低于ET组(均P<0.05)。结论MPN患者的高血压患病率高于总体人群,且呈现明显年轻化特征。PV和ET患者高血压患病具有不同的临床特点。

Myeloproliferative and thrombotic burden and treatment outcome of thrombocythemia and polycythemia patients

Prospective studies indicate that the risk of microvascular and major thrombosis in untreated thrombocythemia in various myeloproliferative neoplasms(MPN-T) is not age dependent and causally related to platelet-mediated thrombosis in early, intermediate and advanced stages of thrombocythemia in MPN-T. If left untreated both microvascular and major thrombosis frequently do occur in MPN-T, but can easily be cured and prevented by low dose aspirin as platelet counts are above 350 × 109/L. The thrombotic risk stratification in the retrospective Bergamo study has been performed in 100 essential thrombocythemia(ET) patients not treated with aspirin thereby overlooking the discovery in 1985 of aspirin responsive platelet-mediated arteriolar and arterial thrombotic tendency in MPN-T disease of ET and polycythemia vera(PV) patients. The Bergamo definition of high thrombotic risk and its persistence in the 2012 International Prognostic Score for ET is based on statistic mystification and not applicable for low and intermediate MPN-T disease burden in ET and PV patients on aspirin. With the advent of molecular screening of MPN patients, MPN-T disease associated with significant leukocytosis, thrombocytosis, constitutional symptoms and/or moderate splenomegaly are candidates for low dose peglyated interferon(Pegasys R, 45 mg/m L once per week or every two weeks) as the first line myeloreductive treatment option in JAK2V617 F mutated MPN-T disease in ET and PV patients. If non-responsive to or side effects induced by IFN, hydroxyurea is the second line myelosuppressive treatment option in JAK2V617 F mutated ET and PV patients with increased MPN-T disease burden.

Overview of dyslipidemia and metabolic syndrome in myeloproliferative neoplasms

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.