HemoCue versus Complete Blood Count for Hemoglobin Measurement in Adults With Vaso-occlusive Crisis Due to Sickle Cell Disease

2013 ◽  
Vol 62 (4) ◽  
pp. S40-S41
Author(s):  
M. Bursey ◽  
K. Curtis ◽  
M. Gibson ◽  
R. Gibson ◽  
H. Xu ◽  
...  
Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4187-4187
Author(s):  
Satish Maharaj ◽  
Simone M Chang ◽  
Ruobing Xue ◽  
Kamila I. Cisak ◽  
Vivek R. Sharma

Abstract Background: Patients with sickle cell disease (SCD) are at increased risk of developing serious infections as a result of functional asplenia and altered humoral immunity. Nevertheless, presenting symptoms of sepsis such as fever and arthralgias are common in SCD and can occur with many sickle cell related conditions, including vaso-occlussive crises (VOC) and may not correlate with true infections. The neutrophil-to-lymphocyte ratio (NLR) is calculated as the absolute neutrophil count divided by the absolute lymphocyte count and represents an easily accessible value that has been found to correlate with inflammation and prognosis in several conditions. Few studies have evaluated NLR as a biomarker in sickle cell disease, and its utility in differentiating infection vs. VOC in patients presenting to the emergency room remains unknown. Method: We conducted a retrospective review of 143 patients with SCD who presented to the emergency department with fever and painful crises. The patients were divided into two categories based on discharge diagnoses - patients with VOC only (n=92) and patients with proven/possible infection (n=51). Inclusion criteria for both groups were patients with SCD, 17 years and older and complete blood count with differential on presentation; patients who had received antibiotics prior to presentation were excluded. Data collected on presentation included genotype, age, gender, complete blood count, hydroxyurea use. Data was analyzed between the two groups using descriptive statistics and receiver-operating characteristic (ROC) curve analysis. Results: Demographics and clinical characteristics are summarized in the Table. The sample included primarily young adult males with 61% on hydroxyurea. Genotype HbSS (73%) was most prevalent followed by HbSC (23%) and HbSβ (4%). The mean Hb was around 8 g/dL. The VOC group had a lower mean white blood cell (WBC) count of 13.6, compare to 17.2 for patients with proven/possible infection. ROC analysis showed that NLR did correlate with infection, with a modest AUC 0.7 [95% CI (0.59-0.77)] that was significant (p=0.0002) when compared to the AUC=0.5 model. Maximum specificity and sensitivity in this sample was achieved with NLR = 4.5 (Specificity 75% and Sensitivity 59%). Conclusion: In this sample, NLR on presentation significantly but only modestly correlated with infection as opposed to VOC. Optimal performance at NLR=4.5 achieved Specificity 75% and Sensitivity 59%. Despite modest performance, given the widespread availability and cost-effectiveness of NLR testing, further study in a larger sample may derive other variables that can combine with NLR to formulate a predictive model to improve care for these patients. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1741-1741
Author(s):  
Katherine Eisenbrown ◽  
Oluwakemi Badaki ◽  
Angela M. Ellison ◽  
Mark Nimmer ◽  
David C. Brousseau

Abstract Background The National Heart, Lung, and Blood Institute Consensus Expert Panel recommends emergency department evaluation for all patients with sickle cell disease who develop a fever. However, it is unclear what recommended components are present in institutional care plans for their patients with sickle cell disease and what variation exists in the evaluation of patients who present with sickle cell disease and fever across institutions. There are few studies evaluating this practice variation and little evidence concerning the appropriate work-up of these children. Our objective was to describe areas where significant practice variation exists in the care of children with sickle cell disease presenting with fever to established sickle cell centers. Methods We undertook a retrospective cross-sectional study of the actual care received at three comprehensive sickle cell centers at pediatric hospitals to understand the diagnostic evaluation, treatment and disposition of children ages 3 months to 21 years presenting to the emergency department with sickle cell disease and fever. Chart reviews were performed on all visits of children presenting with a diagnosis of sickle cell disease to the emergency department of one of the three participating sites between January 1, 2008 and December 31, 2012. All charts were reviewed, and any chart with a documented fever ≥ 38.5°C, either at home or in the emergency department, was included for analysis. Data abstraction included laboratory and radiographic evaluation as well as antibiotic use and disposition. All children pretreated with an antibiotic within the past 24 hours were excluded from the analysis as this could alter the diagnostic evaluation and disposition of these patients. Descriptive statistics were used to determine the percent of children who received a chest radiograph, blood culture, complete blood count, urinalysis, electrolytes, treatment with an antibiotic, and disposition of hospital admission. Due to the large sample sizes, relatively small differences in proportions were determined to be statistically significant; however, differences of less than 10 percent were not considered to necessarily be indicative of clinically meaningful differences in evaluation or treatment, and therefore differences smaller than 10% were reported as similar. Results Analysis to date reveals complete evaluation of 1251 visits [673 at the Children's Hospital of Wisconsin (CHW), 368 at Children's National Medical Center (CNMC), and 210 at Children's Hospital of Philadelphia (CHOP)]. The median age of the children at these visits was 3.4 years (interquartile range of 1.4 - 7.7). Analysis of diagnostic testing revealed approximately 98 percent of patients received a complete blood count and a blood culture, with no difference between sites. Ninety-three percent of patients were treated with an antibiotic, which also showed no meaningful difference across sites. Analysis of disposition revealed significant differences between sites, with 49%, 47%, and 100% of patients admitted to the inpatient units at CHW, CNMC and CHOP, respectively. Likewise, significant differences were seen in obtaining chest radiographs: 81%, 92%, and 29% at CHW, CNMC and CHOP, respectively. The percent of patients who received a urinalysis ranged from a high of 39% at CNMC to a low of 18% at CHOP. Electrolytes were obtained from 3%, 48% and 1% of patients at CHW, CNMC and CHOP, respectively. Conclusion Consistent with NHLBI guidelines, essentially all children with sickle cell disease presenting to the emergency department with fever receive a complete blood count, blood culture and antibiotics. These equal proportions suggest similar treatment guidelines across sites. There is significant variation between sites in the proportion of children who receive a chest x-ray, urinalysis, electrolytes and perhaps most importantly, admission to the hospital. These examples of practice variation may represent potential areas for quality improvement efforts to better define best care practices for children with sickle cell disease presenting to the emergency department for fever. Disclosures: No relevant conflicts of interest to declare.


2005 ◽  
Vol 22 (3) ◽  
pp. 152-159
Author(s):  
Eufemia Jacob ◽  
Christine Miaskowski ◽  
Marilyn Savedra ◽  
Judith E. Beyer ◽  
Marsha Treadwell ◽  
...  

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 10-11
Author(s):  
Satish Maharaj ◽  
Simone Chang ◽  
Karan Seegobin ◽  
Marwan Shaikh ◽  
Kamila I. Cisak

Background: Acute chest syndrome (ACS) frequently complicates sickle cell disease (SCD) and is a leading cause of hospitalization and mortality. Many factors have been implicated in ACS, including infections, thrombosis, fat and pulmonary emboli. However, a clear etiology is not defined in 50% of the cases and ACS is considered a clinical endpoint for different pathogenic processes (Vichinsky et al 2000). The non-specific nature of ACS makes diagnostic tests challenging, and there are no serum tests clinical used to aid diagnosis. Procalcitonin (PCT) is a prohormone of calcitonin and serum PCT rises within hours of an inflammatory stimulus. PCT has clinical utility as a marker of severe systemic inflammation, infection, and sepsis (Becker et al. 2008). Few studies have evaluated PCT as a biomarker for ACS in patients presenting with vaso-occlusive crises (VOC). Two studies have reported no difference in PCT (Biemond et al. 2018 and Stankovic et al 2011), while one study reported higher PCT between ACS and VOC (Patel et al 2014). Methods: We retrospectively reviewed 106 patients with SCD who presented to the emergency department with fever and painful crises during 2015-2019. The patients were divided into two categories based on discharge diagnoses - patients with VOC only (n=88) and patients with ACS (n=18). Inclusion criteria for both groups were patients with SCD, 17 years and older and PCT measurement on presentation. Exclusion criteria were defined as patients who had received empiric antibiotics prior to PCT testing. Data collected on presentation included genotype, age, gender, complete blood count, PCT, creatinine, total bilirubin and hydroxyurea use. Length of stay was recorded. Data was analyzed between the two groups using descriptive statistics and accounting for unequal variances, withp-value set at 0.05 for significance. Results: Demographics and clinical characteristics are summarized in Table 1 (Figure). The sample included primarily adult males (77%), with about two-thirds on hydroxyurea. Genotype HbSS (73.6%) was most prevalent followed by HbSC (22.6%) and HbSβ (3.8%). The ACS group had a higher percentage of HbSS, lower use of hydroxyurea and higher mean bilirubin. Mean PCT for the ACS group was 0.52 ng/mL (range, 0.05-2.04), compared to 0.31 ng/mL (range, 0.02-6.82) in the VOC group; withp=0.084. ROC analysis showed a PCT>0.5ng/mL had 39% sensitivity and 85% specificity for ACS in this sample. Conclusion: In this sample, PCT on presentation was higher in those with ACS compared to VOC, but this difference did not achieve statistical significance. Further study in a larger population would be useful to evaluate this finding. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3521-3521
Author(s):  
Rebekah Shaw ◽  
Sarah Kappa ◽  
Robert Sheppard Nickel

Abstract Background: Hydroxyurea is daily oral medication proven to decrease complications of sickle cell disease (SCD). While concerns have been raised about the safety of hydroxyurea, it is now generally viewed as a well-tolerated medication for SCD. The primary toxicity of hydroxyurea that requires holding of treatment is reversible cytopenia. Due to its classification as a chemotherapeutic agent and safety concerns regarding inappropriate chemotherapy ordering, hydroxyurea can only be ordered by "chemotherapy-certified" providers at some hospitals. At our hospital system, pediatric resident physicians were restricted from ordering hydroxyurea. Instead of being a part of a resident's hospital admission orders, hydroxyurea for inpatients had to be ordered separately by a hematology fellow or attending physician. In June 2016 our hospital changed its policy to allow residents to order hydroxyurea for patients with SCD admitted to the hospital who were already on hydroxyurea at home. We hypothesized that this change in policy to allow residents to order hydroxyurea would increase the proportion of patients with SCD appropriately receiving their home hydroxyurea by hospital day 1. We also hypothesized that this policy change would not result in an increase in the proportion of patients inappropriately receiving hydroxyurea when it should have been held based on the admission complete blood count (CBC). Methods: We conducted a retrospective review of the medical records of a random sample of patients admitted to the hematology service the year before (2015) and the year after (2017) the policy change in 2016. Patients were eligible for study if they were admitted to the hematology service and were taking hydroxyurea as documented by a clinic note within the last three months. Patients were excluded if they were admitted to the intensive care unit or for surgery. Patients were also excluded if discharged on hospital day 0 or 1. Institutional guidelines advise holding hydroxyurea if any of the following: absolute neutrophil count <1250/µL; platelet count <80K/µL; reticulocyte count <100K/µL, unless hemoglobin >8.0 gm/dL. Hydroxyurea was classified as "inappropriately given" if a patient received hydroxyurea despite having an admission CBC value below a hold parameter. Hydroxyurea was classified as "appropriately not given" if a patient did not receive hydroxyurea when having a CBC value below a hold parameter. Patients who were on hydroxyurea who never received hydroxyurea inpatient with CBC values above the hold parameters were classified as "inappropriately not given." Patients admitted in 2015 (before resident ordering) were compared with patients admitted in 2017 (after resident ordering) using a chi-square test or Fisher exact test. Results: In total, 217 hospitalizations of eligible patients were reviewed: 91 before the policy change and 126 after the policy change. Based on the admission CBC, hydroxyurea should have been held for 8 patients. Excluding these patients who should not have received hydroxyurea, patients after the policy change were significantly more likely to have received their home hydroxyurea by hospital day 1: before 62/90 (69%) vs. after 105/119 (88%), p=0.0005. The proportion of patients who inappropriately received hydroxyurea was very low in both groups: before 1/91 (1%) vs. after 3/126 (2%), p=0.64. No serious adverse clinical events occurred from this "inappropriate" administration of hydroxyurea. The figure graphically displays the proportion of patients in the two groups who: appropriately received on hospital day 0/1/2+, inappropriately did not receive, appropriately did not receive, and inappropriately received hydroxyurea. Conclusion: Resident ordering of home hydroxyurea for hospitalized patients with SCD appears to be safe. Policies that permit residents to order hydroxyurea as part of a patient's admission orders can help increase the proportion of patients who receive this important medication while inpatient. Figure. Figure. Disclosures No relevant conflicts of interest to declare.


QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Mohsen Saleh Elalfy ◽  
Mahmoud Adel Kenny ◽  
Fatima Zahrra Abdelkarim Saed ◽  
Fatma Soliman Elsayed Ebeid

Abstract Background Transfusion is critical in the management of sickle cell disease (SCD) complications. The resultant alloimmunization to RBC group antigens is a major complication of allogeneic blood transfusion and generally presents significant challenges in the management of SCD patients. Aim To measure the frequency of the occurrence of the alloimmune markers in sickle cell disease and to investigate its predicators. Subjects and methods This cross-sectional study included 50 children and young adults with SCD, all patients were subjected to detailed medical history thorough clinical examination. Laboratory investigations included complete blood count, markers of hemolysis and serum ferritin. The D, C, c, E, e, M, NJKa and JKb antigens were typed using monoclonal antisera. Fya, Fyb, Jka, Jkb, S, s and Anti Lua antigens were typed by commercially prepared polyclonal anti- human sera. Antibody screening was performed using Ortho screening panel. Results The study Sickle SS was the most common type of SCD representing more than half of the recruited patients. Frequency of antibody positive screening among SCD was 16%. Comparison between SCD patients with positive and those with negative antibody screening showed that higher frequency of positive antibody screening in those with SB+ than those SS and SB0. Patient with positive antibodies screening were significantly older when they received their first blood transfusion and had lower transfusion index (p = 0.037) than those with negative antibodies screening (p=0.013). There were no significant difference between patients with positive and those with negative antibodies screening as regards age, gender, transfusion frequency and as regards comorbidities; painful crisis (p=0.117), stroke (p=0.398), ACS (p=0.363). Conclusion The distribution of the blood group antigens in patients with SCD has important clinical significance especially those who often require regular blood transfusion and who may have developed multiple antibodies. The resultant alloimmunization is serious necessitates early recognition and management.


2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
Ahmad Monabbati ◽  
Sadat Noori ◽  
Akbar Safaei ◽  
Mani Ramzi ◽  
Seyedsajjad Eghbali ◽  
...  

Sickle cell disease (SCD) is a well known hemoglobinopathy with usual manifestations including anemia, hyperbilirubinemia, and vasoocclusive complications. Despite presence of mild splenomegaly in early phase of the disease, lymphadenopathy is not an often finding of SCD. We introduce an undiagnosed case of SCD who presented in third decade of his life with multiple cervical lymphadenopathies and mild splenomegaly persistent for about five years. Histopathologic examination of the resected lymph nodes showed expansion of the mantle cell layers of secondary follicles as well as several monomorphic mantle cell nodules. To rule out possibility of a malignant process involving lymph nodes, an immunohistochemical panel was ordered which was in favor of benign mantle cell hyperplasia. Immunoglobulin gene rearrangement study showed no clonal bands and confirmed benign nature of the process. Respecting mild abnormalities on Complete Blood Count, peripheral blood smear was reviewed revealing some typical sickle red blood cells as well as rare nucleated red blood cells. Solubility test for hemoglobin (HB) S was positive. Hemoglobin electrophoresis confirmed diagnosis of homozygous HbS disease.


2017 ◽  
Vol 2017 ◽  
pp. 1-5 ◽  
Author(s):  
B. F. Faye ◽  
D. Sow ◽  
M. Seck ◽  
N. Dieng ◽  
S. A. Toure ◽  
...  

Introduction. The realization of red cell exchange (RCE) in Africa faces the lack of blood, transfusion safety, and equipment. We evaluated its efficacy and safety in severe complications of sickle cell disease. Patients and Method. Manual partial RCE was performed among sickle cell patients who had severe complications. Efficacy was evaluated by clinical evolution, blood count, and electrophoresis of hemoglobin. Safety was evaluated on adverse effects, infections, and alloimmunization. Results. We performed 166 partial RCE among 44 patients including 41 homozygous (SS) and 2 heterozygous composites SC and 1 S/β0-thalassemia. The mean age was 27.9 years. The sex ratio was 1.58. The regression of symptoms was complete in 100% of persistent vasoocclusive crisis and acute chest syndrome, 56.7% of intermittent priapism, and 30% of stroke. It was partial in 100% of leg ulcers and null in acute priapism. The mean variations of hemoglobin and hematocrit rate after one procedure were, respectively, +1.4 g/dL and +4.4%. That of hemoglobin S after 2 consecutive RCE was −60%. Neither alloimmunization nor viral seroconversion was observed. Conclusion. This work shows the feasibility of manual partial RCE in a low-resource setting and its efficacy and safety during complications of SCD outside of acute priapism.


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