UK Biobank moement tracking data show increased performance as compared to symptoms and genetic and lifestyle factors in identifying prodromal Parkinson's disease in the general population. 15 Polycythemia vera (pol-e-sy-THEE-me-uh VEER-uh) is a type of blood cancer. However, this finding might be another reflection of health and socioeconomic disparities that exist in our society. Finally, EEC growth is based on the ability of erythroid cells from peripheral blood and bone marrow samples in PV to grow in vitro without the addition of EPO.12,13 This unique finding, along with serum EPO levels, forms the basis for a new diagnostic approach,5 but has the disadvantages of expense and limited availability.10. It can quickly turn into a medical emergency. These excess cells thicken your blood, slowing its flow, which may cause serious problems, such as blood clots. Because of increased awareness and earlier diagnosis, the end-organ damage secondary to iron overload is not often seen in clinical practice. Ferrari A, et al. Identifying patients at higher risk of blood culture contamination could alert healthcare providers to take extra precautionary measures to limit contamination in these patients, and thereby prevent associated adverse outcomes. Save my name, email, and website in this browser for the next time I comment. Symptoms such as transient neurologic disturbances may respond to low-dose aspirin therapy. Both patients and health workers can be exposed through phlebotomy to blood from other people, putting them at risk from bloodborne pathogens. After exclusions, the final analytical sample consisted of 11,474 patient admissions with 11,010 negative and 464 contaminated episodes (Table1). Although new diagnostic modalities have been developed, these criteria remain the standard method to diagnose PV.8. 17 Department of Epidemiology, College of Public Health, University of Nebraska Medical Center, Omaha, Nebraska, 2 A blood culture is a test that checks for foreign invaders like bacteria, yeast, and other microorganisms in your blood. Approximately 30 million blood cultures are performed in the United States each year, with most hospitals experiencing contamination rates of 2%3%. We identified patient-specific factors that increase the odds of false-positive blood cultures. 19 THROMBOPROPHYLAXIS All patients should receive daily low-dose aspirin (40 to 100 mg) in the absence of . Hydroxyurea is the first-line agent for cytoreductive therapy in PV. found old age and end-stage renal disease (ESRD) to be significant risk factors for blood culture contamination. Those considered at indeterminate risk are younger than 60 years and have no history of thrombocytosis, but do have cardiovascular or other risk factors.1 Therapy in this group should be individualized, possibly with the addition of agents acting on platelet function or number. 22 Additionally, we could not evaluate the association between ESRD and blood culture contamination, since we did not have a sufficient number of participants with ESRD. BRIAN J. STUART, LT, MC, USNR, AND ANTHONY J. VIERA, LCDR, MC, USNR. How a genetic mutation can cause individuals with normal cholesterol Study participants included 19,255 adult patients who had blood culture(s) performed during a hospital admission between June 2014 and December 2016. Chest pain with shortness of breath can mean a possible pulmonary embolism. Despite the high prevalence of the gene mutation, there is a low and variable clinical penetrance. Blood cultures drawn in emergency departments may be more susceptible to contamination due to staff turnover, the need to collect cultures in critically ill patients during resuscitation, and the time pressure of obtaining cultures before the first dose of antibiotics. Polycythemia Vera: Rapid Evidence Review Blacks had 1.35 higher adjusted odds of having a contaminated blood culture compared to whites (AOR, 1.32; 95% CI, 1.001.81). Patients treated with phlebotomy alone had the same rate of cancer as patients in the control groups.2,14,20, The nonalkylating myelosuppressive agent hydroxyurea is widely used in the treatment of PV, because it is less leukemogenic.22 PVSG data have established this agent to be an effective bone marrow suppressant. Patients admitted from the ED had 1.20 higher adjusted odds of having contaminated blood cultures than those not admitted from ED (AOR, 1.20; 95% CI, 0.961.50). Thrombosis accounts for the majority of morbidity and mortality. First-line treatments, such as low-dose aspirin and goal-directed phlebotomy to a hematocrit level of less than 45% to reduce thrombotic events, improve quality of life and prolong survival. During venipuncture, bacteria on skin fragments can dislodge into the specimen. Doing it right the first time: quality improvement and the contaminant blood culture, Reducing blood culture contamination in community hospital emergency departments: a multicenter evaluation of a quality improvement intervention, Effect of iodophor vs iodine tincture skin preparation on blood culture contamination rate, Practical guidance for clinical microbiology laboratories: a comprehensive update on the problem of blood culture contamination and a discussion of methods for addressing the problem, Reducing blood culture contamination by a simple informational intervention, Factors associated with blood culture contamination in the emergency department: critical illness, end-stage renal disease, and old age. Phlebotomy alone may be the treatment of choice with the goal of reducing the hematocrit . Objective: We hypothesized that phlebotomy overdraw in excess of that requested by the hospital laboratory was a common occurrence, that clinical factors associated with excessive phlebotomy loss would be identified, and that some of these factors are potentially correctable. Your red blood cells are an essential part of your blood. Copyright 2023 American Academy of Family Physicians. Your body uses this oxygen to make the energy needed to perform essential functions. Development of interventions aimed at changing behaviors to improve these conditions may result in improvement in patient care, reduction in healthcare costs, and reduction in bacterial resistance. The annual incidence of PV is 0.01 to 2.61 per 100,000 people, and the prevalence is 0.49 to 46.88 per 100,000.2,3, Median age at diagnosis is 64 years (range = 19 to 95 years), and up to 25% of diagnoses occur before 50 years of age.4,5, Nonmodifiable risk factors for PV include older age, male sex, White race, and European descent.2,6,7, Modifiable risk factors for PV include smoking, obesity, hypertension, diabetes mellitus, and hyperlipidemia.8,9, Patients who have PV typically present with elevated hemoglobin and hematocrit levels found incidentally or after laboratory evaluation for patients' reported symptoms, physical examination findings, or thrombotic/bleeding events.5,10,11, The differential diagnosis of PV includes other myeloproliferative neoplasms and secondary erythrocytosis from smoking, sleep apnea, and androgen use and, rarely, erythropoietin-producing tumors (Table 1).12, Table 2 presents the 2016 World Health Organization's diagnostic criteria for PV.13 A suggested approach to evaluation is summarized in Figure 1.14, Table 3 lists the typical laboratory findings,5 and Table 4 describes the frequency of the most common signs and symptoms at the time of diagnosis.5,10,11, Fatigue, insomnia, and difficulty concentrating are the most common presenting symptoms in patients with PV.11, If PV is suspected after an elevated hemoglobin/hematocrit level is found, Janus kinase 2 (JAK2) V617F mutation and erythropoietin testing should be performed.12, Bone marrow biopsy with fluorescence in situ hybridization testing and karyotyping can be helpful to confirm the diagnosis and to stratify risk.12, The goals of therapy are to improve survival and to improve quality of life by decreasing hematocrit levels, symptom burden, bleeding, and clotting complications.15, No treatments have been shown to reduce the risk of transformation to leukemia or myelofibrosis. Blood Culture: Purpose, Procedure, and Risks The International Council for Standardization in Haematology (ICSH) has amended the RCM assessment, recommending the use of formulas incorporating body surface area, weight, gender, and plasma volume.810 [Level of evidence: C, consensus opinion] A patient with PV could have low oxygen saturation levels, because it is possible to have both PV and an unrelated hypoxic disorder.1 Palpable splenomegaly is an important physical finding and major criterion. Before The test for RCM is a nuclear medicine study involving autologous infusion of radio-labeled red blood cells followed by serial phlebotomy to determine distribution. Because PV is a rare condition, it has been difficult to assemble patients for well-designed, randomized controlled trials with long- term follow-up. The risk of thrombosis was also significantly lower with phlebotomy use (29% vs. 46%).19, All patients should receive daily low-dose aspirin (40 to 100 mg) in the absence of contraindications.20,21, A multicenter randomized controlled trial of 518 patients with a median age of 61 years showed that 100 mg of aspirin used daily over a five-year period significantly reduced the risk of nonfatal myocardial infarction, nonfatal stroke, pulmonary embolism, major venous thrombosis, or death from cardiovascular causes compared with placebo (3.2% vs. 7.9%; number needed to treat = 21 over five years).21, Low-dose aspirin reduces vasomotor symptoms such as headache, erythromelalgia, and pruritus in conjunction with phlebotomy.22, Aspirin use should be avoided in people with extreme thrombocytosis (greater than 1,000 109 per L [1,000 103 per L]) because of increased risk of bleeding from acquired von Willebrand disease.10, Hydroxyurea is the first-line agent for cytoreductive therapy in PV.20, The previously mentioned retrospective cohort study of 820 patients with a median age of 77 years found that use of hydroxyurea was associated with significantly improved survival compared with nonusers (6.0 years vs. 5.3 years). It is characterized by erythrocytosis, often with associated leukocytosis and thrombocytosis. 4. 1.Bloodletting - standards. Polycythemia Vera | AAFP Finally, those considered low risk are younger than 60 years and have no thrombosis-related risk factors. All Rights Reserved. A person viewing it online may make one printout of the material and may use that printout only for his or her personal, non-commercial reference. We used 2 and 2-sample independent t tests to determine associations between the outcome and the covariates. 1 In this study, we sought to determine patient-specific factors contributing to blood culture contamination in hospitalized patients. Often the condition is found during a blood test done for another reason. Trends in blood culture contamination: a College of American Pathologists Q-Tracks study of 356 institutions. reports research funding from Magnolia Medical Technologies. Phlebotomy overdraw in the neonatal intensive care nursery Course director(s), planning committee, faculty, and all others who are in a position to control the content of . We used ICU stay during admission as a proxy for severity of clinical status and observed higher odds of blood contamination in patients with ICU stay. In general, blood cultures obtained from the peripheral vein were drawn by trained phlebotomists, and blood cultures from central venous catheters were drawn by nurses. Similar to predictive models assisting clinicians with the recognition of potentially bacteremic patients, Inclusion in an NLM database does not imply endorsement of, or agreement with, The liver, pancreas, joints, heart, skin, and pituitary gland are the most commonly involved organs. All patients who had blood culture(s) performed at any time during an admission were included in the study population. Major diagnostic criteria include increased RCM, normal oxygen saturation, and the presence of splenomegaly. CDC is one of the federal agencies responsible for promoting the safety of the U.S. blood supply. Nearly half of the patients in both groups were male, and 80% were white race. Up to 10% of the data were missing for smoking status, and up to 30% were missing data for alcohol status. But proper medical care can help ease signs, symptoms and complications of this disease. Consultation with a hematologist is appropriate to aid in diagnosis, and serum EPO levels and bone marrow biopsy should be considered if available. Data Sources: We searched Essential Evidence Plus, Cochrane Database of Systematic Reviews, and PubMed with the key words polycythemia vera, myeloproliferative neoplasms, epidemiology, diagnosis, treatment, therapy, prognosis, risk stratification, management, pregnancy, randomized controlled trial, meta-analysis, systematic review, aspirin, hydroxyurea, phlebotomy, interferon-alpha, busulfan, ruxolitinib, and JAK2 kinase inhibitors. The PVSG and Gruppo Italiano Studio Policitemia (GISP) are two prospective trials that have unearthed a therapeutic dilemma regarding the two basic treatment approachesphlebotomy alone and phlebotomy plus myelosuppressive agents. More-specific symptoms of polycythemia vera include: Make an appointment with your doctor if you have signs or symptoms of polycythemia vera. This series is appropriate for phlebotomists, phlebotomy managers, phlebotomy educators, and other patient-care staff involved in specimen collection. Patient education: Hereditary hemochromatosis (Beyond the Basics) Erythromelalgia is rare, occurring in approximately 3 percent of patients with PV. See permissionsforcopyrightquestions and/or permission requests. 2019. Women are often diagnosed later than men, likely because of menstrual blood loss. As a result, patients with COPD might be more prone to blood culture contamination. Ferri FF. Taking the time to identify risk factors can not only provide a safer experience for the patient, but also help the phlebotomist prevent any potential accidents or injuries during the procedure. What is Phlebotomy? History, Risks, and Side Effects Sign up for free and stay up to date on research advancements, health tips, current health topics, and expertise on managing health. Analysis of vasovagal syncope in the blood collection room in - Nature Best practice in phlebotomy and blood collection Treating hemochromatosis can help relieve symptoms of tiredness, stomach pain and skin darkening. Hemochromatosis is a common heritable disorder characterized by the progressive accumulation of iron attributable to increased absorption from dietary sources. It likely will not improve preexisting diabetes, arthralgias, cirrhosis, and . The results of this study showed that patients admitted from the emergency department were more likely to have contaminated blood cultures. The number of blood collection tubes and waiting time as risk factors of VVS. Polycythemia Vera Management and Challenges in the Community Health Genetic testing for the hereditary hemochromatosis genes should be offered after 18 years of age to first-degree relatives of patients with the condition. Agents under investigation include interferon alfa-2b, anagrelide, and aspirin. Klucher JM, Davis K, Lakkad M, Painter JT, Dare RK. Risk Factors in Phlebotomy WHO guidelines on drawing blood Cuthbert D, et al. The mainstay of treatment for PV is phlebotomy, which is aimed at reducing hyperviscosity by decreasing the venous hematocrit level to less than 45 percent (0.45) in white men and 42 percent (0.42) in blacks and women.1,14,18 The PVSG reported the best median survival, 12.6 years, for this type of treatment.14 Some features of using phlebotomy alone are attractive, primarily because it is a simple procedure without many risks, except for the eventual development of iron deficiency.8 Some experts have cast doubt on the PVSG findings regarding median survival, noting that up to 50 percent of patients treated with phlebotomy alone had to switch to other treatments by the fifth year.19 The PVSG found a statistically significant increase in the number of thrombotic events within the first three years of initiating treatment, compared with the use of myelosuppressive agents.14 After this period, however, the rate of thrombosis remained the same for both treatment approaches. A novel molecular pathway to explain how a mutation in the gene ACTA2 can cause individuals in their 30s -- with normal cholesterol levels and no other risk factors -- to develop coronary artery . Your email address will not be published. Polycythemia vera. https://www.nhlbi.nih.gov/health-topics/polycythemia-vera. Annals of Hematology. This groups generally shorter life expectancy lessens the threat of eventual iatrogenic malignancy. There are four types of hereditary hemochromatosis, which are categorized by the specific gene mutation involved (Table 1).1,5,6, Homozygous C282Y and heterozygous C282Y/H63D mutations of the HFE gene (iron regulatory protein) on chromosome 6 are responsible for up to 95% of hereditary hemochromatosis cases (type 1).1,3, Hereditary hemochromatosis types 2 to 4 comprise the small percentage of remaining cases and are caused by non- HFE mutations.1,5, Hereditary hemochromatosis is the most common inherited disorder among people of northern European ancestry.1,3 The United States, Europe, and Australia have a similar disease prevalence of one case per 200 to 400 people, with the highest prevalence in those of Irish and Scandinavian ancestry.1,3,5, The hereditary hemochromatosis mutation is rare in people of Asian, African, Hispanic, and Pacific Islander ancestry.7, Despite the high prevalence of the gene mutation, there is a low and variable clinical penetrance, with up to 25% of people with C282Y homozygosity being clinically asymptomatic.4,8, The clinical manifestations of iron overload typically develop in the 40s or 50s.1, The disease is equally prevalent in men and women. A common initial presentation is an asymptomatic patient with mildly elevated liver enzymes who is subsequently found to have elevated serum ferritin and transferrin saturation. Patients discharged from the emergency department were excluded; however, patients who were initially treated in the emergency department and subsequently admitted to the hospital were included. Treatment includes phlebotomy with the possible addition of myelosuppressive agents based on a risk-stratified approach. Blood culture contamination results in substantial adverse effects such as prolonged hospital stay and inappropriate antibiotic use. Therapeutic Phlebotomy - Medical Clinical Policy Bulletins | Aetna Phlebotomy can resolve or markedly improve weakness, fatigue, and lethargy; and darkening of the skin. Alahmadi YM, Aldeyab MA, McElnay JC, et al. Once the blood draw is complete, pressure should be applied firm and long enough so that the draw sites don't bleed for long. Serum erythropoietin (EPO), bone marrow histopathology and karyotype, and the presence of endogenous erythroid colonies (EEC) have been proposed as diagnostic tests for PV. 2019; doi:10.2147/JBM.S189922. If you already have one of these conditions, phlebotomy may slow the progression of the disease. All Rights Reserved. Covariates associated with the outcome variable in crude analyses at = 0.1 were included in a multivariable logistic regression model. Patients in a critical condition had higher odds of having contaminated blood cultures, and this has been observed in other studies. However, this effect disappeared after three years, and rates of thrombosis thereafter were equivalent.8,14,19 Unfortunately, prospective data have revealed the mutagenic potential of myelosuppressive agents such as 32P, with a relative risk for malignancy of 2.3 to four times that of the control groups after about six years of treatment. Chang et al Overview. HHS Vulnerability Disclosure, Help Men are more likely to get polycythemia vera, but women tend to get the disease at younger ages. Basilic veins should be avoided unless there are no other antecubital options. Accessibility Data were analyzed to evaluate risk factors for blood culture contamination using logistic regression. Without treatment, polycythemia vera can be life-threatening. It causes your bone marrow to make too many red blood cells. References. We observed a significant association between COPD and contamination of blood cultures (AOR, 1.67; 95% CI, 1.202.34) as was also noted in the study by Klucher et al. Hereditary hemochromatosis is usually diagnosed in the 40s or 50s. Contaminated blood cultures can be difficult to interpret and can have a negative impact on patient management by incurring treatment delays, exposing patients to unnecessary and inappropriate antibiotics, unnecessarily extending hospital stay, and requiring additional testing and consultation. 2018; doi:10.1038/s41408-017-0042-7. Note: ED, emergency department; ICU, intensive care unit; SD, standard deviation. Written by Angela Nelson Medically Reviewed by Jennifer Robinson, MD on June 21, 2021 What to Expect Risks and Side Effects History of Phlebotomy Phlebotomy is. Many people with polycythemia vera don't have noticeable signs or symptoms. Hereditary Hemochromatosis: Rapid Evidence Review In these patients, ruxolitinib achieved better hematocrit control and spleen volume improvement (decrease in volume greater than 35%) compared with other single-drug alternatives selected by the investigator, such as hydroxyurea (tolerable doses), pegylated interferon-alfa, pipobroman (not available in the United States), anagrelide (Agrylin), immunomodulators, or no medication (busulfan was prohibited) over a 32-week period (21% with ruxolitinib vs. 0.9% with other single-drug alternatives; P < .001; number needed to treat = 5).30, Immunosuppressive adverse effects made herpes zoster infection more likely (6% with ruxolitinib vs. 0% with other single-drug alternatives).30, Pruritus may be present in up to 68% of patients and is severe in up to 15% of patients.10, First-line therapy includes aspirin, antihistamines, and paroxetine (Paxil).22,31,32, Other treatment options include ruxolitinib, interferon-alfa, and narrow-band ultraviolet B phototherapy.3335, PV during pregnancy is rare, with a prevalence of less than 0.03 per 100,000.36, A multidisciplinary team, including an obstetrician, maternal-fetal medicine specialist, and hematologist, is recommended.37, Potentially teratogenic medications should be stopped in men and women at least three months before conception.36,37, All pregnant patients who have PV should receive low-dose aspirin, avoid iron supplementation in the absence of actual depletion, maintain gestational age-appropriate hematocrit levels, and be treated with enoxaparin (Lovenox) for six weeks postpartum if no contraindications occur.36, Interferon-alfa is the drug of choice for cytoreductive therapy if needed.36, Follow-up appointments every three to six months are recommended for routine history, physical examination, and complete blood count.15, Poor prognostic features include age older than 60 years, history of thrombosis, leukocytosis, high JAK2 burden, abnormal karyotype, and established cardiovascular risk factors such as smoking, hypertension, diabetes, obesity, and hyperlipidemia.5,9,38, Smoking cessation decreases the risk of thrombosis.39, Without treatment, death typically occurs within two years, mostly from thrombotic events.40, Median survival of patients with PV using standard-of-care treatment, including aspirin and hydroxyurea, is 13.5 years.4, For those diagnosed before 60 years of age, median survival is 24 years.4, The risk of blast transformation to acute myeloid leukemia or myelodysplastic syndrome over 15 years is 5.5% to 18.7%.4,41, Progression to myelofibrosis over 15 years is 6% to 14%.4,41 A practical tool to calculate survival has been developed and is shown in Table 6.5, This article updates a previous article on this topic by Stuart and Viera.1. In some cases, it may even reverse it. Copyright 2023 American Academy of Family Physicians. knowledge of patient-specific factors associated with contamination could help in formulating predictive equations to better identify patients at higher risk of blood culture contamination. The true consequences of false-positive results. A patient information handout on polycythemia vera, written by the authors of this article, is provided on page 2146. Clinical and economic impact of contaminated blood cultures within the hospital setting. All people with polycythemia vera should receive phlebotomy with a goal hematocrit level of less than 45%. A blood culture was considered contaminated if skin-residing organism(s) were identified in 1 of the 2 or more blood-culture sets. The 20-min whole blood clotting test (20WBCT) is a simple, inexpensive bedside test which may be performed to identify incoagulable blood of patients envenomed by snakes such as Echis spp., which induce a consumptive coagulopathy (Benjamin et al., 2018; From: Advances in Protein Chemistry and Structural Biology, 2022 View all Topics Add to Mendeley It usually develops slowly, and you might have it for years without knowing. Limited-quality randomized controlled trials, Systematic review and meta-analysis of lower-quality studies, Systematic review and meta-analysis of cohort studies, Other myeloproliferative neoplasms (essential thrombocytosis, primary myelofibrosis, chronic myelogenous leukemia, myelodysplastic syndrome), Primary familial and congenital polycythemia (, Cardiopulmonary disease (chronic hypoxia, left-to-right shunt), Erythropoietin-producing tumors (e.g., hepatocellular carcinoma, renal cell carcinoma, hemangioblastoma, pheochromocytoma, uterine leiomyomata), Iatrogenic (testosterone, anabolic steroids, erythropoietin, blood doping), Renal disease (renal artery stenosis, hydronephrosis, transplant), Sleep apnea, obesity-hypoventilation syndrome, pickwickian syndrome, Smoking, chronic carbon monoxide poisoning, 184 (18.4 g per dL); (151 [15.1 g per dL] to 265 [26.5 g per dL]), 10.4 (10,400 per L); (3 [3,000 per L] to 171.6 [171,600 per L]), (1) Need for phlebotomy to keep hematocrit level < 45% after 3 months of hydroxyurea (at least 2 g per day), (1) Absolute neutrophil count of 1.0 10, Leukocyte count at presentation 15 10. 8600 Rockville Pike A comprehensive review1 reported the incidence of PV to be 2.3 per 100,000 persons per year. Patients Clinical and Sociodemographic Characteristics by Blood Culture Status. Diagnosis and Treatment of Thrombocythemia in Myeloproliferative Disorders Wearable movement-tracking data identify Parkinson's disease years 1 The most common hemochromatosis genotype is homozygosity for HFE C282Y, although the role of HFE protein in the pathogenesis of the iron overload has not been clearly elucidated. All analyses were conducted using SAS version 9.4 software (SAS Institute, Cary, NC). Advertising revenue supports our not-for-profit mission. If the patient complains of any of these symptoms during venipuncture, withdraw the needle immediately.5 Draw specimens for culture before administering antibiotics. 14 Mayo Clinic. Received 2021 Nov 3; Revised 2022 Jan 15; Accepted 2022 Jan 18. Additionally, we observed an association between BMI and blood culture contamination with a higher odds of contamination for every additional unit of BMI (AOR, 1.01; 95% CI, 1.001.02), which was also demonstrated by Klucher et al.