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Journal of Stroke and Cerebrovascular... Jan 2022Patients with polycythemia, either primary or secondary, are at elevated risk for thrombotic complications, including stroke. We aimed to investigate the clinical and...
PURPOSE
Patients with polycythemia, either primary or secondary, are at elevated risk for thrombotic complications, including stroke. We aimed to investigate the clinical and radiological characteristics of cerebrovascular disease (CVD) in polycythemia, and describe other neurologic manifestations.
METHODOLOGY
We conducted a cross-sectional study of patients diagnosed with polycythemia between 2014 and 2019 at a tertiary care center and collected relevant medical data with a special focus on cerebrovascular disease and neurologic manifestations. We performed descriptive and inferential analyses. We have also described and analyzed the available neuroimaging features.
RESULTS
We analyzed data from 56 patients. 20 patients (35.7%) had ischemic stroke. The incidence of CVD was higher in those with primary polycythemia (43%) than in those with secondary polycythemia (8%). The most common subtype of stroke was large vessel disease, and the most common arterial territory was the anterior circulation. There was no statistically significant difference in the hematocrit level between those with or without CVD. Neuroimaging revealed multiple large vessel intracranial stenoses on MR Angiography, and hyperdense vessels on plain CT. Other neurologic manifestations included headache, seizures, dizziness, visual symptoms and papilledema, and these were significantly more common in primary polycythemia.
CONCLUSIONS
CVD is common in patients with polycythemia. The most common type observed was large vessel occlusion, predominantly in the anterior circulation. In stroke patients, multiple vessel stenosis and hyperdense vessels may be clues to polycythemia.
Topics: Cerebrovascular Disorders; Cross-Sectional Studies; Humans; India; Magnetic Resonance Angiography; Polycythemia; Tertiary Care Centers
PubMed: 34785446
DOI: 10.1016/j.jstrokecerebrovasdis.2021.106167 -
JPMA. the Journal of the Pakistan... Mar 2021To calculate the incidence of post-transplant erythrocytosis, and to assess the response to treatment.
OBJECTIVE
To calculate the incidence of post-transplant erythrocytosis, and to assess the response to treatment.
METHODS
The prospective study was conducted from April 2016 to April 2018 at the Department of Nephrology, Bahria International Hospital, Lahore, Pakistan, and comprised patients undergoing renal transplantation who were evaluated and followed up for 12 months. Patients having haemoglobin levels ≥17gm/dl were labelled as having polycythemia. Data was analysed using SPSS 21.
RESULTS
Of the 94 total patients, 69(73.4%) were enrolled. During follow-up, 2(2.9%) of them died, and, thus, the final sample stood at 67(71.3%); 57 (85%) males and 10(15%) females. The mean age of the sample was 32.6±8.8 years. Overall, 19(28.4%) patients developed polycythemia and they were either given angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers. Of these 19 patients, 11(57.8 %) responded to the treatment, while 8(42.1%) required phlebotomy. Further, 3(15.7%) patients required one phlebotomy, while 5(26.3%) who had glomerular filtration rate >30% had to have repeated phlebotomy.
CONCLUSIONS
The incidence of post-transplant erythrocytosis was significantly high at 28.4%.
Topics: Adult; Angiotensin-Converting Enzyme Inhibitors; Female; Humans; Kidney Transplantation; Male; Pakistan; Polycythemia; Prospective Studies; Young Adult
PubMed: 34057941
DOI: 10.47391/JPMA.1140 -
Annals of Family Medicine 2023Gender-affirming hormone therapy (GAHT) is safe overall, with few adverse effects. One potential effect from using testosterone for GAHT is an increase in hemoglobin...
PURPOSE
Gender-affirming hormone therapy (GAHT) is safe overall, with few adverse effects. One potential effect from using testosterone for GAHT is an increase in hemoglobin and/or hematocrit, known as secondary erythrocytosis. Current guidelines recommend monitoring hemoglobin or hematocrit routinely in the first year, some as frequently as every 3 months, which can create barriers to care. Our study explored the incidence of erythrocytosis in the first 20 months of testosterone therapy among people receiving gender-affirming care.
METHODS
This is a descriptive fixed cohort study of hematocrit and hemoglobin data from the charts of 282 people taking testosterone for GAHT.
RESULTS
During the first 20 months of testosterone therapy, the cumulative incidence of hematocrit >50.4% was 12.6%, hematocrit >52% was 1.0%, and hematocrit >54% was 0.6%. All people were taking injectable testosterone cypionate, with a median dose of 100 mg weekly.
CONCLUSION
Severe erythrocytosis (hematocrit >54%) is a rare outcome of gender-affirming testosterone therapy. Clinical recommendations should reconsider the need for routine frequent erythrocytosis screening within the first year of testosterone therapy for patients who prefer to minimize laboratory draws.
Topics: Humans; Polycythemia; Cohort Studies; Testosterone; Drug-Related Side Effects and Adverse Reactions; Hemoglobins
PubMed: 37748907
DOI: 10.1370/afm.3018 -
Consensus diagnostic criteria and monitoring of twin anemia-polycythemia sequence: Delphi procedure.Ultrasound in Obstetrics & Gynecology :... Sep 2020Twin anemia-polycythemia sequence (TAPS) is associated with increased perinatal morbidity and mortality. Inconsistencies in the diagnostic criteria for TAPS exist, which...
OBJECTIVES
Twin anemia-polycythemia sequence (TAPS) is associated with increased perinatal morbidity and mortality. Inconsistencies in the diagnostic criteria for TAPS exist, which hinder the ability to establish robust evidence-based management or monitoring protocols. The main aim of this study was to determine, by expert consensus using a Delphi procedure, the key diagnostic features and optimal monitoring approach for TAPS.
METHODS
A Delphi process was conducted among an international panel of experts on TAPS. Panel members were provided with a list of literature-based parameters for diagnosing and monitoring TAPS. They were asked to rate the importance of the parameters on a five-point Likert scale. Consensus was sought to determine the cut-off values for accepted parameters, as well as parameters used in the monitoring of and assessment of outcome in twin pregnancy complicated by TAPS.
RESULTS
A total of 132 experts were approached. Fifty experts joined the first round, of whom 33 (66%) completed all three rounds. There was agreement that the monitoring interval for the development of TAPS should be every 2 weeks and that the severity should be assessed antenatally using a classification system based on middle cerebral artery (MCA) peak systolic velocity (PSV), but there was no agreement on the gestational age at which to start monitoring. Once the diagnosis of TAPS is made, monitoring should be scheduled weekly. For the antenatal diagnosis of TAPS, the combination of MCA-PSV ≥ 1.5 MoM in the anemic twin and ≤ 0.8 MoM in the polycythemic twin was agreed. Alternatively, MCA-PSV discordance ≥ 1 MoM can be used to diagnose TAPS. Postnatally, hemoglobin difference ≥ 8 g/dL and intertwin reticulocyte ratio ≥ 1.7 were agreed criteria for diagnosis of TAPS. There was no agreement on the cut-off of MCA-PSV or its discordance for prenatal intervention. The panel agreed on prioritizing perinatal and long-term survival outcomes in follow-up studies.
CONCLUSIONS
Consensus-based diagnostic features of TAPS, as well as cut-off values for the parameters involved, were agreed upon by a panel of experts. Future studies are needed to validate these diagnostic features before they can be used in clinical trials of interventions. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.
Topics: Adult; Anemia; Delphi Technique; Female; Fetofetal Transfusion; Gestational Age; Humans; Polycythemia; Pregnancy; Pregnancy, Twin; Prenatal Diagnosis
PubMed: 31605505
DOI: 10.1002/uog.21882 -
Medicine Jan 2020We have been examining the Comprehensive Health Check of the Fukushima Health Management Survey of residents of 13 municipalities who were forced by the government to... (Observational Study)
Observational Study
We have been examining the Comprehensive Health Check of the Fukushima Health Management Survey of residents of 13 municipalities who were forced by the government to evacuate due to the 2011 Great East Japan Earthquake (GEJE). Our findings showed that evacuation is a risk factor for polycythemia and suggested that experiencing an unprecedented disaster and exposure to chronic stress due to evacuation might be a cause of polycythemia.We analyzed the relationship between the prevalence of polycythemia and the following factors observed in the Mental Health and Lifestyle Survey in an observational study with a cross-sectional design: traumatic symptoms, depression status, socioeconomic factors such as residential environment, and working situation after the GEJE. Target population of the survey included men and women who were at least 15 years of age and who lived in the evacuation zones specified by the government. Participants analyzed consisted of 29,474 persons (12,379 men and 16,888 women) who had participated in both the 2011 Comprehensive Health Check and Mental Health and Lifestyle Survey from June 2011 through March 2012.The prevalence of polycythemia was not associated with mental states associated with traumatic symptoms (Post-Traumatic Stress Disorder Checklist Scale ≥ 44) and depression status (Kessler 6-item Scale ≥ 13). Furthermore, multivariate analysis showed that there was a tendency for males to develop polycythemia, with characteristics such as being aged 65 years and older, highly educated, obese (body mass index ≥ 25), hypertensive, diabetic, having liver dysfunction, and a smoker being significantly related to the prevalence of polycythemia.Our findings conclusively demonstrated that polycythemia was not significantly related to psychological factors, but was significantly related to the onset of lifestyle-related disease after the GEJE.
Topics: Adult; Aged; Aged, 80 and over; Causality; Comorbidity; Earthquakes; Female; Fukushima Nuclear Accident; Humans; Japan; Life Style; Male; Middle Aged; Polycythemia; Prevalence; Tsunamis; Young Adult
PubMed: 31895781
DOI: 10.1097/MD.0000000000018486 -
Ethiopian Journal of Health Sciences Nov 2023Surgical treatment has transformed the course and outcome of congenital heart defects in high-income countries, but children with congenital heart diseases in...
BACKGROUND
Surgical treatment has transformed the course and outcome of congenital heart defects in high-income countries, but children with congenital heart diseases in sub-Saharan Africa, where access to cardiac surgery is limited, often experience the natural course of untreated lesions and their complications. The objective of this study was to determine the prevalence of hematologic derangements among Ethiopian children with unoperated cyanoticcongenital heart diseases, to identify factors associated with coagulopathy in this population, and to describe how these complications are managed in this setting.
METHODS
In this single-center cross-sectional study, we prospectively collected clinical and demographic data from children (<18 years) with cyanotic congenital heart diseases. Blood samples were collected to measure hematologic parameters. Polycythemia was defined as hematocrit >50% and thrombocytopenia as <150,000 per microliter.
RESULTS
Among 70 children recruited, the overall prevalence of polycythemia and thrombocytopenia was 63% (n=44) and 26% (n=18), respectively. On multivariate logistic regression analysis, hematocrit ≥65% (p-value=.024), and oxygen saturation <85% (p-value=.018) were independently associated with moderate or severe thrombocytopenia. Thirty-one (44%) patients had undergone therapeutic phlebotomy, and 84% (26/31) of these patients received iron supplementation.
CONCLUSION
We report a high prevalence of polycythemia and thrombocytopenia in Ethiopian children with untreated cyanotic congenital heart diseases. There was variable implementation of iron supplementation and therapeutic phlebotomy, highlighting the need to optimize supportive management strategies in this population to mitigate the risk of life-threatening complications.
Topics: Humans; Ethiopia; Female; Heart Defects, Congenital; Male; Cross-Sectional Studies; Polycythemia; Child, Preschool; Infant; Child; Thrombocytopenia; Prevalence; Hematocrit; Cyanosis; Adolescent; Prospective Studies; Phlebotomy
PubMed: 38784485
DOI: 10.4314/ejhs.v33i6.5 -
Blood May 2021Polycythemia and pulmonary hypertension are 2 human diseases for which better therapies are needed. Upregulation of hypoxia-inducible factor-2α (HIF-2α) and its target...
Polycythemia and pulmonary hypertension are 2 human diseases for which better therapies are needed. Upregulation of hypoxia-inducible factor-2α (HIF-2α) and its target genes, erythropoietin (EPO) and endothelin-1, causes polycythemia and pulmonary hypertension in patients with Chuvash polycythemia who are homozygous for the R200W mutation in the von Hippel Lindau (VHL) gene and in a murine mouse model of Chuvash polycythemia that bears the same homozygous VhlR200W mutation. Moreover, the aged VhlR200W mice developed pulmonary fibrosis, most likely due to the increased expression of Cxcl-12, another Hif-2α target. Patients with mutations in iron regulatory protein 1 (IRP1) also develop polycythemia, and Irp1-knockout (Irp1-KO) mice exhibit polycythemia, pulmonary hypertension, and cardiac fibrosis attributable to translational derepression of Hif-2α, and the resultant high expression of the Hif-2α targets EPO, endothelin-1, and Cxcl-12. In this study, we inactivated Hif-2α with the second-generation allosteric HIF-2α inhibitor MK-6482 in VhlR200W, Irp1-KO, and double-mutant VhlR200W;Irp1-KO mice. MK-6482 treatment decreased EPO production and reversed polycythemia in all 3 mouse models. Drug treatment also decreased right ventricular pressure and mitigated pulmonary hypertension in VhlR200W, Irp1-KO, and VhlR200W;Irp1-KO mice to near normal wild-type levels and normalized the movement of the cardiac interventricular septum in VhlR200Wmice. MK-6482 treatment reduced the increased expression of Cxcl-12, which, in association with CXCR4, mediates fibrocyte influx into the lungs, potentially causing pulmonary fibrosis. Our results suggest that oral intake of MK-6482 could represent a new approach to treatment of patients with polycythemia, pulmonary hypertension, pulmonary fibrosis, and complications caused by elevated expression of HIF-2α.
Topics: Animals; Basic Helix-Loop-Helix Transcription Factors; Endothelin-1; Erythropoietin; Female; Gene Expression Regulation; Hypertension, Pulmonary; Iron Regulatory Protein 1; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Polycythemia; Sulfones; Von Hippel-Lindau Tumor Suppressor Protein
PubMed: 33512384
DOI: 10.1182/blood.2020009138 -
Expert Review of Respiratory Medicine Apr 2022Although polycythemia has been considered a common adverse event in COPD, anemia is reported more often and has gained more importance than polycythemia over the last... (Review)
Review
INTRODUCTION
Although polycythemia has been considered a common adverse event in COPD, anemia is reported more often and has gained more importance than polycythemia over the last thirty years.
AREAS COVERED
Factors considered to be associated with the development of anemia in COPD have included: Aging and kidney dysfunction with erythropoietin deficiency and bone marrow suppression due to uremic toxins; heart failure (HF), often encountered in COPD and accompanied by anemia in one-third of the cases; Low-grade chronic inflammation, directly suppressing bone marrow and diminishing iron absorption and utilization via increased hepcidin levels; long-term oxygen therapy (LTOT), ameliorating chronic hypoxia, and most important, RAS inhibitors, which are widely used for the comorbidities associated with COPD (hypertension, HF, CKD, diabetes) and have previously been shown to lower hematocrit values or cause anemia in various clinical conditions.
EXPERT OPINION
Introduction of LTOT in COPD and especially the established use of RAS inhibitors form the basis for the shift from polycythemia to anemia in COPD. Interestingly, when the SGLT2 inhibitors are introduced for cardiorenal protection in COPD, one could anticipate correction of anemia or even reemergence of polycythemia, since this new class of drugs can augment erythropoietin secretion and increase hematocrit values.
Topics: Anemia; Antihypertensive Agents; Erythropoietin; Humans; Polycythemia; Pulmonary Disease, Chronic Obstructive; Renin-Angiotensin System
PubMed: 35212601
DOI: 10.1080/17476348.2022.2045958 -
American Journal of Respiratory Cell... Jan 2020Desquamative interstitial pneumonia (DIP) is a rare, smoking-related, diffuse parenchymal lung disease characterized by marked accumulation of alveolar macrophages (AMs)...
Desquamative interstitial pneumonia (DIP) is a rare, smoking-related, diffuse parenchymal lung disease characterized by marked accumulation of alveolar macrophages (AMs) and emphysema, without extensive fibrosis or neutrophilic inflammation. Because smoking increases expression of pulmonary GM-CSF (granulocyte/macrophage-colony stimulating factor) and GM-CSF stimulates proliferation and activation of AMs, we hypothesized that chronic exposure of mice to increased pulmonary GM-CSF may recapitulate DIP. Wild-type (WT) mice were subjected to inhaled cigarette smoke exposure for 16 months, and AM numbers and pulmonary GM-CSF mRNA levels were measured. After demonstrating that smoke inhalation increased pulmonary GM-CSF in WT mice, transgenic mice overexpressing pulmonary GM-CSF (SPC-GM-CSF) were used to determine the effects of chronic exposure to increased pulmonary GM-CSF (without smoke inhalation) on accumulation and activation of AMs, pulmonary matrix metalloproteinase (MMP) expression and activity, lung histopathology, development of polycythemia, and survival. In WT mice, smoke exposure markedly increased pulmonary GM-CSF and AM accumulation. In unexposed SPC-GM-CSF mice, AMs were spontaneously activated as shown by phosphorylation of STAT5 (signal inducer and activator of transcription 5) and accumulated progressively with involvement of 84% (interquartile range, 55-90%) of the lung parenchyma by 10 months of age. Histopathologic features also included scattered multinucleated giant cells, alveolar epithelial cell hyperplasia, and mild alveolar wall thickening. SPC-GM-CSF mice had increased pulmonary MMP-9 and MMP-12 levels, spontaneously developed emphysema and secondary polycythemia, and had increased mortality compared with WT mice. Results show cigarette smoke increased pulmonary GM-CSF and AM proliferation, and chronically increased pulmonary GM-CSF recapitulated the cardinal features of DIP, including AM accumulation, emphysema, secondary polycythemia, and increased mortality in mice. These observations suggest pulmonary GM-CSF may be involved in the pathogenesis of DIP.
Topics: Animals; Emphysema; Epithelial Cells; Genetic Diseases, Inborn; Granulocyte-Macrophage Colony-Stimulating Factor; Hyperplasia; Lung; Lung Diseases, Interstitial; Macrophages, Alveolar; Matrix Metalloproteinase 12; Matrix Metalloproteinase 9; Mice; Mice, Inbred C57BL; Mice, Transgenic; Polycythemia; Pulmonary Alveoli; STAT5 Transcription Factor; Smoking
PubMed: 31310562
DOI: 10.1165/rcmb.2018-0294OC -
Combinatorial Chemistry & High... 2021Hyperviscosity of blood secondary to polycythemia results in increased resistance to blood flow and decrease in delivery of oxygen.
BACKGROUND
Hyperviscosity of blood secondary to polycythemia results in increased resistance to blood flow and decrease in delivery of oxygen.
OBJECTIVE
To evaluate whether serum endocan, NSE and IMA levels can be compared in terms of endothelial injury/ dysfunction and neuronal damage in term neonates with polycythemia who underwent PET.
METHODS
38 symptomatic polycythemic newborns having PET and 38 healthy newborns were included in the study. Blood samples for endocan, NSE and IMA were taken at only postnatal 24 hours of age in the control group and in polycytemia group just before PET, at 24 and 72 hours after PET.
RESULTS
The polycythemia group had higher serum endocan(1073,4 ± 644,8 vs. 378,8 ± 95,9ng/ml; p<0.05), IMA(1,32 ± 0,34 vs.0,601 ± 0,095absorbance unit; p<0.05) and NSE(44,7 ± 4,3 vs. 26,91 ± 7,12μg/l; p<0.05) levels than control group before the PET procedure. At 24 hours after PET, IMA(0,656 ± 0,07 vs. 0,601 ± 0,095absorbance unit; p<0.05) and endocan(510,9 ± 228,6 vs. 378,8 ± 95,9ng/ml; p<0.05) levels were closer to the control group, being still statistically significant higher. NSE levels decreased to control group levels having no difference between the PET and control groups at 24 hours after PET (28,98 ± 6,5 vs. 26,91 ± 7,12μg/l; p>0.05). At 72 hours after PET the polycythemia and control groups did not differ statistically for IMA, endocan and NSE levels (p>0.05).
CONCLUSION
Serum endocan and IMA levels can be used as a biomarker for endothelial damage/ dysfunction and tissue hypoxia in infants with symptomatic polycytemia.
Topics: Biomarkers; Exchange Transfusion, Whole Blood; Humans; Infant, Newborn; Neoplasm Proteins; Phosphopyruvate Hydratase; Polycythemia; Proteoglycans; Serum Albumin, Human
PubMed: 33109054
DOI: 10.2174/1386207323999200820163525