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 Uterine myomas are the most common benign gynecological soft tissue tumors in women but polycythemia associated with these tumors is rare.Polycythemia is significantly associated with risk of venous thrombo...BACKGROUND Uterine myomas are the most common benign gynecological soft tissue tumors in women but polycythemia associated with these tumors is rare.Polycythemia is significantly associated with risk of venous thromboembolism(VTE),which is further increased in case of a large pelvic mass and obesity.Here we report the surgical challenges in the case of an obese patient with a giant fibromatous uterus and associated polycythemia.CASE SUMMARY A 42-year-old obese woman was referred to our department for treatment for a huge fibromatous uterus.She suffered of pelvic pressure and abdominal distension and reported severe dyspnea from several months.Laboratory analyses including hemoglobin(Hb)19.2 g/dL and hematocrit(Hct)59.7%were indicative of polycythemia.Arterial blood gas analysis showed arterial oxygen pressure(pO2)of 81.5 mmHg.Abdominal computed tomography confirmed a giant fibromatous uterus measuring 28.2 cm×17 cm×25 cm.To reduce the thromboembolic risk,the patient underwent low molecular weight heparin,phlebotomy twice before surgery,and we opted for a laparoscopic hysterectomy.The uterus weighed 5400 g and the histology confirmed a diagnosis of leiomyoma.We did not find increased erythropoietin levels in the supernatant of the myomatous uterine tissue.There were no complications.On postoperative day 1,the Hb and the Hct levels normalized to 13.3 g/dL and 41.7%,respectively.Arterial blood gas analysis after surgery showed a pO2 of 144.7 mmHg.These results suggested the diagnosis of myomatous erythrocytosis syndrome.The patient was discharged on the second postoperative day in very good condition with no symptoms.CONCLUSION We believe this is the first reported laparoscopic hysterectomy in a patient with a giant uterus,polycythemia and obesity.Multiple VTE risk factors warranted a laparoscopic approach.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
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-mediat...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.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
BACKGROUND:Polycythemia vera is defined as a chronic myeloproliferative disorder characterized by increased red blood cell count.There have been no reports on mesenteric thrombosis resulting from iatrogenic polycythem...BACKGROUND:Polycythemia vera is defined as a chronic myeloproliferative disorder characterized by increased red blood cell count.There have been no reports on mesenteric thrombosis resulting from iatrogenic polycythemia.METHODS:We present a patient with a history of non-small cell lung cancer undergoing maintenance oral chemotherapy on tarceva and adjunctive use of procrit.The patient presented to our emergency department with an acute abdomen and was found to have ischemic bowel from unmonitored procrit,which lead to hyperviscosity of blood and mesenteric infarction.RESULTS:The patient remained intubated with ventilator support.He refused a tracheostomy.He continued on feeding through the J port of the nasojejunal tube.His white cell count,and hematocrit and creatinine levels remained normal.Procrit use and chemotherapy were not restarted.He was transferred to a subacute nursing facility for further treatment.CONCLUSIONS:Procrit and other erythropoiesis stimulating drugs can cause significant morbidity and mortality with an increased risk of cardiovascular events,gastrointestinal bleeding,thromboembolism and stroke.This case report suggests that without closely monitoring hematocrit levels,epoetin may also be associated with an increased risk of mesenteric infarction.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 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
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
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
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
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
Objective: To investigate the curative effect of Tongmai Huoxue Decoction combined with warm needling on the treatment of High altitude polycythemia (HAPC), and to analyze the changes of hemorheology and antioxidant a...Objective: To investigate the curative effect of Tongmai Huoxue Decoction combined with warm needling on the treatment of High altitude polycythemia (HAPC), and to analyze the changes of hemorheology and antioxidant ability before and after treatment. Methods: A total of 90 HAPC patients were randomly divided into control group and observation group. The control group was treated with low molecular weight dextran and captopril. On the basis of this, the observation group was supplemented with Tongmai Huoxue Decoction combined with warm needling. Hemorheology and antioxidant markers were measured before and after treatment in all subjects. Results: Before treatment, there were no significant differences in the hemorheology indexes between the control group and the observation group. After treatment, the two groups of hemorheology related indicators were significantly lower than those before treatment, including whole blood low shear reduction viscosity, whole blood high shear reduction viscosity, plasma viscosity, Hb, Hct and RBC. The levels of the observation group were significantly lower than that of the control group. Before treatment, there was no significant difference in antioxidant indexes between the control group and the observation group. After treatment, the levels of serum superoxide dismutase (SOD) and glutathione (GSH) in the two groups were higher than those in the pre-treatment, while the content of malondialdehyde (MDA) was lower than that before treatment. The levels of serum SOD and GSH in the observation group were higher than those in the control group, and the MDA content was lower than that in the control group. Conclusion: Routine treatment of Western medicine combined with Tongmai Huoxue Decoction joint warm needling method may reduce hemoglobin, improve hemorheology and improve antioxidant ability in HAPC patients, and it may be one of the potential effective drugs for adjuvant therapy of HAPC.展开更多
Either dengue fever or diabetic ketoacidosis can cause the high hemoglobin concentration due to intravascular volume loss.When both entities were found together as in our case,the hemoglobin concentration could access...Either dengue fever or diabetic ketoacidosis can cause the high hemoglobin concentration due to intravascular volume loss.When both entities were found together as in our case,the hemoglobin concentration could access the strikingly high level until either of them could not contribute it.She was a Thai woman with 46 years of age presenting with fever,fatigue and severe sore throat for a few days.The physical examination showed she had mild dyspnea and oral thrush,the temperature was 36.4 degree Celsius,and her pulse rate was 102/min.Her blood tests showed:Hb 18.3 g%,WBC 9,850/mm^(3),atypical lymphocyte 10%,platelet 16,000/mm^(3),MCV 89.2 fl,Dengue NS1 antigen-positive,dengue IgG-positive,IgM-weakly positive,KOH preparation of curd from buccal mucosa-positive for budding yeasts and pseudo-hyphae,blood sugar 442 mg%,triglyceride 578 mg%,Na 122.8 mEq/L,K 6.28 mEq/L,Cl 90.6 mEq/L,CO_(2)9.2 mEq/L,blood ketone 53.6 mmol/L,lactate 4.5 mmol/L,pH 7.257,pCO_(2)27.4 mmHg,HCO_(3)11.9 mmo/L,BUN 22.9 mg%,creatinine 0.64 mg%,AST 375 U/L,ALT 224 U/L,alkaline phosphatase 151 U/L.She was diagnosed as having diabetes with diabetic ketoacidosis,dengue fever and polycythemia.She was immediately treated with intravenous fluid therapy to correct the metabolic acidosis,electrolyte imbalance and dehydration,insulin for hyperglycemia,clotrimazole for oral thrush and platelet transfusion.Her high hemoglobin level was dramatically lowered until became normal within one day of therapy whereas she also recovered from DKA although the platelet was progressively lowered every day.It suggested that transient polycythemia was solely contributed by DKA that was triggered by dengue fever hence DKA from dengue fever should be added in the list of unusual causes of transient polycythemia.展开更多
Objective To investigate the effects of simple hypobaric hypoxia on parameters of hematology and blood rheology in order to establish a rat model of simulated high altitude polycythemia(HAPC) for the study of pathophy...Objective To investigate the effects of simple hypobaric hypoxia on parameters of hematology and blood rheology in order to establish a rat model of simulated high altitude polycythemia(HAPC) for the study of pathophysiologic mechanisms and medical prevention and treatment of HAPC.Methods Fortyeight male Wistar rats were randomly divided into three normal control groups and three hypoxia model groups.Normal control group rats were bred in normoxia conditions,and hypoxia group rats were subjected to hypoxic exposure for 8 hours per day at simulated 5 500 m high altitude in a hypobaric chamber.After hypoxic exposure for 2,4,12 weeks,one group of normal control and hypoxia model rats were killed and blood was collected,respectively.Then parameters of erythrocyte and blood rheology were examined.Results Mucous membrane of hypoxia model rats showed obviously cyanosis after 2 weeks hypoxic exposure.Hemoglobin concentration of hypoxia model rats were beyond 210 g/L after 2 weeks,4 weeks and 12 weeks hypoxia exposure and significantly increased than that of normal control rats respectively.Besides,RBC counts,hematocrit,whole blood viscosity,erythrocyte aggregation index of hypoxia model rats were all notably higher than those of normal control rats respectively.Conclusion A rat model of high altitude polycythemia can be rapidly established by hypobaric hypoxia exposure at simulated 5 500 m high altitude for 8 hours daily.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
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
Twin anemia polycythemia sequence (TAPS) is a chronic form of unbalanced feto-fetal transfusion through minuscule placental anastomoses in monochorionic twin pregnancies, leading to anemia in the donor twin and polycy...Twin anemia polycythemia sequence (TAPS) is a chronic form of unbalanced feto-fetal transfusion through minuscule placental anastomoses in monochorionic twin pregnancies, leading to anemia in the donor twin and polycythemia in the recipient twin. TAPS can occur spontaneously in up to 5% of monochorionic twins or can arise in 2%-16% of cases after incomplete laser surgery for twin-twin transfusion syndrome. TAPS can develop across the entire second and third trimester. Antenatal diagnosis for TAPS is reached via Doppler measurement of the fetal middle cerebral artery peak systolic velocity, showing an increased velocity in the donor, combined with a decreased velocity in the recipient. Treatment options for TAPS include expectant management, preterm delivery, intrauterine blood transfusion with or without a partial exchange transfusion, fetoscopic laser surgery and selective feticide. The best treatment option is unclear and is currently being investigated in an international multicenter randomized trial (the TAPS trial). Spontaneous fetal demise occurs in 5%-11% of TAPS twins, more often in donors (8%-18%) than in recipients (2%-5%). Severe long-term neurodevelopmental impairment is seen in 9% of TAPS twins, with donors having an increased risk for cognitive impairment and hearing problems (15%).展开更多
Objective: To explore the protective effects of Tibetan medicine Zuo-Mu-A Decoction(佐木阿汤, ZMAD) on the blood parameters and myocardium of high altitude polycythemia(HAPC) model rats. Methods: Forty male Wistar rat...Objective: To explore the protective effects of Tibetan medicine Zuo-Mu-A Decoction(佐木阿汤, ZMAD) on the blood parameters and myocardium of high altitude polycythemia(HAPC) model rats. Methods: Forty male Wistar rats were randomly divided into 4 groups by a random number table, including the normal, model, Rhodiola rosea L.(RRL) and ZMAD groups(10 in each group). Every group was raised in Lhasa to create a HAPC model except the normal group. After modeling, rats in the RRL and the ZMAD groups were administered intragastrically with RRL(20 mL/kg) and ZMAD(7.5 mL/kg) once a day for 2 months, respectively; for the normal and the model groups, 5 mL of distilled water was administered intragastrically instead of decoction. Then routine blood and hematologic rheology parameters were taken, levels of erythropoietin and 8-hydroxy-2'-deoxyguanosine(8-OHd G) were tested, and ultrastructural change in the left ventricular myocardium was observed using transmission electron microscopy. Results: Compared with the model group, ZMAD significantly reduced the red blood cell count, hemoglobin levels, whole blood viscosity at low/middle shear rates, plasma viscosity, erythrocyte electrophoretic time, erythropoietin and 8-OHd G levels, and also increased the erythrocyte deformation index(P<0.05). There was no difference in all results between the RRL and the ZMAD groups. The cardiac muscle fibers were well-protected, mitochondrial matrix swelled mildly and ultrastructure changes were less prominent in the ZMAD group compared with the model group. Conclusion: ZMAD has significant protective effects on the blood parameters against HAPC, and also has the beneficial effect in protecting against myocardial injury.展开更多
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.
文摘BACKGROUND Uterine myomas are the most common benign gynecological soft tissue tumors in women but polycythemia associated with these tumors is rare.Polycythemia is significantly associated with risk of venous thromboembolism(VTE),which is further increased in case of a large pelvic mass and obesity.Here we report the surgical challenges in the case of an obese patient with a giant fibromatous uterus and associated polycythemia.CASE SUMMARY A 42-year-old obese woman was referred to our department for treatment for a huge fibromatous uterus.She suffered of pelvic pressure and abdominal distension and reported severe dyspnea from several months.Laboratory analyses including hemoglobin(Hb)19.2 g/dL and hematocrit(Hct)59.7%were indicative of polycythemia.Arterial blood gas analysis showed arterial oxygen pressure(pO2)of 81.5 mmHg.Abdominal computed tomography confirmed a giant fibromatous uterus measuring 28.2 cm×17 cm×25 cm.To reduce the thromboembolic risk,the patient underwent low molecular weight heparin,phlebotomy twice before surgery,and we opted for a laparoscopic hysterectomy.The uterus weighed 5400 g and the histology confirmed a diagnosis of leiomyoma.We did not find increased erythropoietin levels in the supernatant of the myomatous uterine tissue.There were no complications.On postoperative day 1,the Hb and the Hct levels normalized to 13.3 g/dL and 41.7%,respectively.Arterial blood gas analysis after surgery showed a pO2 of 144.7 mmHg.These results suggested the diagnosis of myomatous erythrocytosis syndrome.The patient was discharged on the second postoperative day in very good condition with no symptoms.CONCLUSION We believe this is the first reported laparoscopic hysterectomy in a patient with a giant uterus,polycythemia and obesity.Multiple VTE risk factors warranted a laparoscopic approach.
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.
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BACKGROUND:Polycythemia vera is defined as a chronic myeloproliferative disorder characterized by increased red blood cell count.There have been no reports on mesenteric thrombosis resulting from iatrogenic polycythemia.METHODS:We present a patient with a history of non-small cell lung cancer undergoing maintenance oral chemotherapy on tarceva and adjunctive use of procrit.The patient presented to our emergency department with an acute abdomen and was found to have ischemic bowel from unmonitored procrit,which lead to hyperviscosity of blood and mesenteric infarction.RESULTS:The patient remained intubated with ventilator support.He refused a tracheostomy.He continued on feeding through the J port of the nasojejunal tube.His white cell count,and hematocrit and creatinine levels remained normal.Procrit use and chemotherapy were not restarted.He was transferred to a subacute nursing facility for further treatment.CONCLUSIONS:Procrit and other erythropoiesis stimulating drugs can cause significant morbidity and mortality with an increased risk of cardiovascular events,gastrointestinal bleeding,thromboembolism and stroke.This case report suggests that without closely monitoring hematocrit levels,epoetin may also be associated with an increased risk of mesenteric infarction.
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 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.
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.
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Objective: To investigate the curative effect of Tongmai Huoxue Decoction combined with warm needling on the treatment of High altitude polycythemia (HAPC), and to analyze the changes of hemorheology and antioxidant ability before and after treatment. Methods: A total of 90 HAPC patients were randomly divided into control group and observation group. The control group was treated with low molecular weight dextran and captopril. On the basis of this, the observation group was supplemented with Tongmai Huoxue Decoction combined with warm needling. Hemorheology and antioxidant markers were measured before and after treatment in all subjects. Results: Before treatment, there were no significant differences in the hemorheology indexes between the control group and the observation group. After treatment, the two groups of hemorheology related indicators were significantly lower than those before treatment, including whole blood low shear reduction viscosity, whole blood high shear reduction viscosity, plasma viscosity, Hb, Hct and RBC. The levels of the observation group were significantly lower than that of the control group. Before treatment, there was no significant difference in antioxidant indexes between the control group and the observation group. After treatment, the levels of serum superoxide dismutase (SOD) and glutathione (GSH) in the two groups were higher than those in the pre-treatment, while the content of malondialdehyde (MDA) was lower than that before treatment. The levels of serum SOD and GSH in the observation group were higher than those in the control group, and the MDA content was lower than that in the control group. Conclusion: Routine treatment of Western medicine combined with Tongmai Huoxue Decoction joint warm needling method may reduce hemoglobin, improve hemorheology and improve antioxidant ability in HAPC patients, and it may be one of the potential effective drugs for adjuvant therapy of HAPC.
文摘Either dengue fever or diabetic ketoacidosis can cause the high hemoglobin concentration due to intravascular volume loss.When both entities were found together as in our case,the hemoglobin concentration could access the strikingly high level until either of them could not contribute it.She was a Thai woman with 46 years of age presenting with fever,fatigue and severe sore throat for a few days.The physical examination showed she had mild dyspnea and oral thrush,the temperature was 36.4 degree Celsius,and her pulse rate was 102/min.Her blood tests showed:Hb 18.3 g%,WBC 9,850/mm^(3),atypical lymphocyte 10%,platelet 16,000/mm^(3),MCV 89.2 fl,Dengue NS1 antigen-positive,dengue IgG-positive,IgM-weakly positive,KOH preparation of curd from buccal mucosa-positive for budding yeasts and pseudo-hyphae,blood sugar 442 mg%,triglyceride 578 mg%,Na 122.8 mEq/L,K 6.28 mEq/L,Cl 90.6 mEq/L,CO_(2)9.2 mEq/L,blood ketone 53.6 mmol/L,lactate 4.5 mmol/L,pH 7.257,pCO_(2)27.4 mmHg,HCO_(3)11.9 mmo/L,BUN 22.9 mg%,creatinine 0.64 mg%,AST 375 U/L,ALT 224 U/L,alkaline phosphatase 151 U/L.She was diagnosed as having diabetes with diabetic ketoacidosis,dengue fever and polycythemia.She was immediately treated with intravenous fluid therapy to correct the metabolic acidosis,electrolyte imbalance and dehydration,insulin for hyperglycemia,clotrimazole for oral thrush and platelet transfusion.Her high hemoglobin level was dramatically lowered until became normal within one day of therapy whereas she also recovered from DKA although the platelet was progressively lowered every day.It suggested that transient polycythemia was solely contributed by DKA that was triggered by dengue fever hence DKA from dengue fever should be added in the list of unusual causes of transient polycythemia.
基金supported by grants from National New Drug Research and Development of Key Project (2011ZXJ09105-05B)the National Natural Science Foundation(81171870,81471812)
文摘Objective To investigate the effects of simple hypobaric hypoxia on parameters of hematology and blood rheology in order to establish a rat model of simulated high altitude polycythemia(HAPC) for the study of pathophysiologic mechanisms and medical prevention and treatment of HAPC.Methods Fortyeight male Wistar rats were randomly divided into three normal control groups and three hypoxia model groups.Normal control group rats were bred in normoxia conditions,and hypoxia group rats were subjected to hypoxic exposure for 8 hours per day at simulated 5 500 m high altitude in a hypobaric chamber.After hypoxic exposure for 2,4,12 weeks,one group of normal control and hypoxia model rats were killed and blood was collected,respectively.Then parameters of erythrocyte and blood rheology were examined.Results Mucous membrane of hypoxia model rats showed obviously cyanosis after 2 weeks hypoxic exposure.Hemoglobin concentration of hypoxia model rats were beyond 210 g/L after 2 weeks,4 weeks and 12 weeks hypoxia exposure and significantly increased than that of normal control rats respectively.Besides,RBC counts,hematocrit,whole blood viscosity,erythrocyte aggregation index of hypoxia model rats were all notably higher than those of normal control rats respectively.Conclusion A rat model of high altitude polycythemia can be rapidly established by hypobaric hypoxia exposure at simulated 5 500 m high altitude for 8 hours daily.
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Twin anemia polycythemia sequence (TAPS) is a chronic form of unbalanced feto-fetal transfusion through minuscule placental anastomoses in monochorionic twin pregnancies, leading to anemia in the donor twin and polycythemia in the recipient twin. TAPS can occur spontaneously in up to 5% of monochorionic twins or can arise in 2%-16% of cases after incomplete laser surgery for twin-twin transfusion syndrome. TAPS can develop across the entire second and third trimester. Antenatal diagnosis for TAPS is reached via Doppler measurement of the fetal middle cerebral artery peak systolic velocity, showing an increased velocity in the donor, combined with a decreased velocity in the recipient. Treatment options for TAPS include expectant management, preterm delivery, intrauterine blood transfusion with or without a partial exchange transfusion, fetoscopic laser surgery and selective feticide. The best treatment option is unclear and is currently being investigated in an international multicenter randomized trial (the TAPS trial). Spontaneous fetal demise occurs in 5%-11% of TAPS twins, more often in donors (8%-18%) than in recipients (2%-5%). Severe long-term neurodevelopmental impairment is seen in 9% of TAPS twins, with donors having an increased risk for cognitive impairment and hearing problems (15%).
基金Supported by the National Natural Science Foundation of China(No.81260567)
文摘Objective: To explore the protective effects of Tibetan medicine Zuo-Mu-A Decoction(佐木阿汤, ZMAD) on the blood parameters and myocardium of high altitude polycythemia(HAPC) model rats. Methods: Forty male Wistar rats were randomly divided into 4 groups by a random number table, including the normal, model, Rhodiola rosea L.(RRL) and ZMAD groups(10 in each group). Every group was raised in Lhasa to create a HAPC model except the normal group. After modeling, rats in the RRL and the ZMAD groups were administered intragastrically with RRL(20 mL/kg) and ZMAD(7.5 mL/kg) once a day for 2 months, respectively; for the normal and the model groups, 5 mL of distilled water was administered intragastrically instead of decoction. Then routine blood and hematologic rheology parameters were taken, levels of erythropoietin and 8-hydroxy-2'-deoxyguanosine(8-OHd G) were tested, and ultrastructural change in the left ventricular myocardium was observed using transmission electron microscopy. Results: Compared with the model group, ZMAD significantly reduced the red blood cell count, hemoglobin levels, whole blood viscosity at low/middle shear rates, plasma viscosity, erythrocyte electrophoretic time, erythropoietin and 8-OHd G levels, and also increased the erythrocyte deformation index(P<0.05). There was no difference in all results between the RRL and the ZMAD groups. The cardiac muscle fibers were well-protected, mitochondrial matrix swelled mildly and ultrastructure changes were less prominent in the ZMAD group compared with the model group. Conclusion: ZMAD has significant protective effects on the blood parameters against HAPC, and also has the beneficial effect in protecting against myocardial injury.
