A Comparison of Hematologic Parameters in Patients with Sickle Cell Disease Undergoing Red Cell Exchange Using Terumo BCT Spectra Optia and COBE Spectra Apheresis Systems

Abstract Introduction: Therapeutic red blood cell exchange (RBCX) is a process by which diseased red cells are replaced by healthy donor red blood cells. In patients with sickle cell disease, RBCX has been used to treat acute stroke, severe acute chest syndrome, multiorgan failure, priapism and splenic sequestration. It is also being used more commonly in the prevention of vaso-occlusive pain crises and for stroke prophylaxis for patients considered to be at high risk of stroke based on abnormal transcranial Doppler flow rates. In May 2014, we transitioned from the COBE Spectra apheresis system (Terumo BCT) to the next-generation Spectra Optia apheresis system (Terumo BCT) for all red cell exchange transfusions performed on our patients with sickle cell disease. A previous small study (n = 33 RBCX transfusions) compared the two instruments (Caridian BCT) and showed no difference between exchange volumes, processing time, pre and post-exchange hematocrit and HbS levels (Perseghin et al. Transfusion and Apheresis Science, 2013). However, other clinical parameters such as changes in white blood cell count and platelet counts were not examined. In order to determine if there were any differences in hematologic parameters between the 2 apheresis instruments, we measured the differences between pre and post-exchange HbS levels, white blood cell count (WBC), hematocrit (Hct), and platelet counts for all of our adult patients who underwent RBCX transfusions on both instruments. Methods: This was a single institution, retrospective chart review of all adult patients with sickle cell disease (HbSS n=17, HbS/β0 thalassemia n = 1, HbSC n =1) who underwent routine, monthly RBCX at Children's Hospital and Research Center Oakland between November 2013 and February 2015. Indications for RBCX included a risk of stroke or a history of stroke, acute chest syndrome, or renal failure. All patients received RBCX transfusions on the COBE Spectra prior to May 2014 and were then transitioned to Spectra Optia in May 2014. Pre and post-exchange transfusion HbS levels, WBC, Hct, and platelet counts were measured for each procedure. All exchange transfusions were non-emergent and well tolerated. Statistical analyses using the student's t test and rank sum test were performed with Stata 14.0 software (College Station, Texas). Results: A total of 19 adult patients (mean average age 25 years old) underwent 153 red blood cell exchange transfusions (Spectra Optia n =87, COBE Spectra n = 66). There was a small increase in mean hematocrit percentage for both instruments (28.33 + 4.3 % to 29 + 3.06 % and 28.16 + 4.29% to 29.2 + 3.04% on the Spectra Optia and COBE Spectra, respectively). All other post-RBCX parameters decreased. The mean changes are shown in Table I. The HbS percent decreased from a combined mean of 39.86 + 12.11 % to 20.26 + 8.43 % for both instruments. The WBC decreased from a combined mean of 12.72 + 3.13 x 1000/ mm3 to 8.67 + 2.15 x 1000/ mm3. The platelet count decreased from a combined mean of 368.82 + 125.75 x 1000/ mm3 to 191.01 + 62.78 x 1000/ mm3. The mean parameter values for each patient correlated with the mean changes for each instrument based on statistical analysis using both the student's t test and rank sum test. Conclusions: The starting hematologic values were similar between the two instruments. There was no statistical difference between the raw pre and post-RBCX HbS, Hct, WBC, or platelet values or the mean changes in these parameters between the COBE Spectra and Spectra Optia instruments. There was also no statistical difference in the mean changes between the pre and post RBCX hematologic parameters amongst the 19 patients. Both instruments allowed for effective reduction in HbS percentage with comparable decreases in WBC and platelet counts while maintaining adequate hematocrit values for all 19 adult patients with sickle cell disease. Table 1. Mean changes between Pre-RBCX and Post-RBCX. Instrument Change in % HbS Change in WBC x1000/mm3 Change in % Hct Change in platelet count x1000/mm3 Spectra Optia N 84 87 87 85 Mean -20.28 -4.01 0.67 -172.44 SD 7.02 2.55 2.86 76.98 COBE Spectra N 66 66 66 66 Mean -19.18 -4.12 1.09 -178.02 SD 7.89 2.26 2.57 81.02 p-value 0.37 0.78 0.34 0.67 There are 3 missing values for HbS and 2 missing values for platelet count for Spectra Optia. SD = standard deviation, WBC = white blood cell count, Hct = hematocrit Disclosures No relevant conflicts of interest to declare.

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White Blood Cell Count as a Predictor of the Severity of Sickle Cell Disease During Pregnancy

Obstetrical & Gynecological Survey ◽

10.1097/01.ogx.0000298314.63671.93 ◽

2008 ◽

Vol 63 (1) ◽

pp. 4-5

Author(s):

Michail Litos ◽

Ippokratis Sarris ◽

Susan Bewley ◽

Paul Seed ◽

Iheanyi Okpala ◽

...

Keyword(s):

Sickle Cell Disease ◽

Blood Cell ◽

Cell Count ◽

Sickle Cell ◽

White Blood Cell Count ◽

White Blood Cell ◽

Cell Disease ◽

Blood Cell Count

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White blood cell count as a predictor of the severity of sickle cell disease during pregnancy

European Journal of Obstetrics & Gynecology and Reproductive Biology ◽

10.1016/j.ejogrb.2006.08.009 ◽

2007 ◽

Vol 133 (2) ◽

pp. 169-172 ◽

Cited By ~ 13

Author(s):

Michail Litos ◽

Ippokratis Sarris ◽

Susan Bewley ◽

Paul Seed ◽

Iheanyi Okpala ◽

...

Keyword(s):

Sickle Cell Disease ◽

Blood Cell ◽

Cell Count ◽

Sickle Cell ◽

White Blood Cell Count ◽

White Blood Cell ◽

Cell Disease ◽

Blood Cell Count

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Non-crisis related pain occurs in adult patients with sickle cell disease despite chronic red blood cell exchange transfusion therapy

Transfusion and Apheresis Science ◽

10.1016/j.transci.2021.103304 ◽

2021 ◽

pp. 103304

Author(s):

Susanna A. Curtis ◽

Balbuena-Merle Raisa ◽

John D. Roberts ◽

Jeanne E. Hendrickson ◽

Joanna Starrels ◽

...

Keyword(s):

Sickle Cell Disease ◽

Blood Cell ◽

Sickle Cell ◽

Red Blood Cell ◽

Exchange Transfusion ◽

Adult Patients ◽

Cell Disease ◽

Transfusion Therapy

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Surgical Site Infections in a Tertiary Referral Obstetric and Gynecologic Clinic Center in Istanbul and Review of the Literature

Gynecology Obstetrics and Reproductive Medicine ◽

10.21613/gorm.2017.717 ◽

2018 ◽

Vol 24 (1) ◽

pp. 37

Author(s):

Hale Goksever Celik ◽

Engin Celik ◽

Selin Dikmen ◽

Merve Konal ◽

Ali Gedikbasi

Keyword(s):

Blood Cell ◽

Cell Count ◽

White Blood Cell Count ◽

White Blood Cell ◽

Psychological Health ◽

Wound Care ◽

Surgical Site Infections ◽

Abdominal Hysterectomy ◽

Blood Cell Count ◽

The Mean

Objective: The present study aimed to define characteristics of the patients who were readmitted with the diagnosis of surgical site infections after gynecologic or obstetric procedures and management of these patients. We also reviewed the literature in this context.Study design: We examined 120 patients with surgical site infections that had been hospitalized and managed medically and/or surgically between April 2014 and April 2015. Characteristics of the patients were recorded and analyzed.Results: The mean age of the patients was 33.4±11.8 years. The patients were readmitted for surgical site infections on the mean of 9.6±5.4 days after the first operation. The most frequent procedures resulted with SSI were cesarean delivery, abdominal hysterectomy and vaginal birth. When patients were compared according to these procedures, there were statistically significant differences regarding age, gravida, parity, preoperative white blood cell count, postoperative white blood cell count and antibiotics usage.Conclusion: Combining evidence-based surgical site infections prevention practices and clinician and patient cooperation will result in reduction in surgical site infections incidence following obstetric and gynecologic procedures. Because of economic burden and threat to the physical and psychological health of the patients, these modifiable risks should be recognized and surgical site infections should be minimized. After surgical site infections occurred, diagnosis and proper management with antibiotics and wound care with debridement and secondary suturing is important.

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Red Blood Cell Adhesion in Adult Patients with Sickle Cell Disease, at Baseline and with Pain, Measured on SCD Biochip Microfluidic Assay

Blood ◽

10.1182/blood-2018-99-115586 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 2386-2386

Author(s):

Alexandra Boye-Doe ◽

Jane Little

Keyword(s):

Cell Adhesion ◽

Sickle Cell Disease ◽

Blood Cell ◽

Sickle Cell ◽

Red Blood Cell ◽

Research Funding ◽

Adult Patients ◽

Cell Disease ◽

University Hospitals ◽

Western Reserve

Abstract Red Blood Cell Adhesion in Adult Patients with Sickle Cell Disease, at Baseline and with Pain, Measured on SCD Biochip Microfluidic Assay Alexandra Boye-Doe1, Erina Quinn1, Charlotte Yuan1, Umut A. Gurkan, PhD1, and Jane A. Little, MD2 1Case Western Reserve University, Cleveland, OH; 2Division of Hematology/Oncology, Case Western Reserve University/ University Hospitals Seidman Cancer Center, Cleveland, OH Background: Despite being monogenic, sickle cell disease (SCD) has a variable phenotype, in which clinical complications and manifestations evolve as patients age. In children, pain generally resolves between crises, whereas adults may experience acute, chronic, or acute-on-chronic pain. We have taken a multifaceted approach to characterize adhesion and inflammation during self-identified pain episodes in adults with SCD in order to better understand pain syndromes in adults and the potential for more specifically targeted therapies. In this pilot study, we assessed changes in red blood cell (RBC) adhesion to the subendothelial protein laminin (LN) during crisis in adults with SCD self-reporting for pain crisis on whom we had baseline adhesion data from a routine clinic visit. Methods: Surplus blood from patients' routine bloodwork was used. Crisis samples were collected from patients at the Acute Care Clinic or Emergency Department at University Hospitals Cleveland Medical Center (UHCMC) when patients presented for management of pain. Baseline samples were collected during routine visits to the Sickle Cell Clinic. This study was approved by the IRB at UHCMC. Within a 24-hour period, RBC adhesion to LN was quantitated by microscopy after passage of unprocessed whole blood through a LN-coated microfluidic adhesion assay, the SCD biochip [1]. Samples were analyzed for hemoglobin (Hb) phenotype by high-performance liquid chromatography (HPLC) in the clinical lab. Correlative clinical data, including, baseline lab values, and medical history, were obtained from the patients' medical records and used to characterize our results. Data from people with multiple samples were used as median values. Results: Blood samples from 19 unselected patients with sickle cell hemoglobin SS (HbSS) were obtained at crisis, and compared with baseline samples obtained from 2014 to 2018 (n = 67 samples). 2 groups were identified: Group 1 with increased adhesion (>25% rise from baseline, n= 10) during crisis, and Group 2 with decreased adhesion (>25% fall from baseline, n=8) or no change (<25% change) (n=1) during crisis (Fig. 1). Time between a patient's initial crisis event and when they presented for pain management varied, possibly affecting observed adhesion. Nonetheless, patients showing an increased adhesion in crisis also showed a decrease in Hb (p = 0.039) between their baseline analyses and the crisis visit. A decrease in Hb may associate with increased adhesion, if the latter contributes to crisis-related hemolysis. However, other markers of hemolysis did not change. By contrast, patients with decreasing RBC adhesion showed a decrease in absolute reticulocyte count (ARC, p = 0.019) at their crisis visit. Statistical analysis of HbS, HbA, and HbF levels n = 64 showed no significant change between baseline or crises. Discussion: A decrease in adhesion during crisis may reflect the presence of sickled RBCs that adhere preferentially to other basement membrane proteins such as fibronectin or thrombospondin. In addition, inflammatory white blood cell adhesion, possibly central to vaso-occlusion, is not evaluated in this study. We are currently examining cytokine and monocyte profiles from people with SCD presenting for management of pain, so that we may better understand inflammatory mediators of pain. Our data are the first to try to understand RBC adhesion in people with SCD who present for management of pain, whether this as an isolated or common event for that person. We found that unselected adults with SCD who presented for management of pain had heterogeneous changes in RBC adhesion, which may reflect differences in underlying mechanism. The pathophysiologic heterogeneity of pain in adults with SCD will be important to understand as anti-adhesion therapies are being developed and adopted clinically. Disclosures Little: Doris Duke Charitable Foundations: Research Funding; NHLBI: Research Funding; Hemex: Patents & Royalties: Patent, no honoraria; PCORI: Research Funding.

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‘The white blood cell always eat the red’: how Jamaicans with sickle cell disease understand their illness

Ethnicity and Health ◽

10.1080/13557858.2015.1028522 ◽

2015 ◽

Vol 21 (2) ◽

pp. 103-117 ◽

Cited By ~ 2

Author(s):

Moji Anderson ◽

Monika Asnani

Keyword(s):

Sickle Cell Disease ◽

Blood Cell ◽

Sickle Cell ◽

White Blood Cell ◽

Cell Disease

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Barriers to using molecularly typed minority red blood cell donors in support of chronically transfused adult patients with sickle cell disease

Transfusion ◽

10.1111/trf.13037 ◽

2015 ◽

Vol 55 (6pt2) ◽

pp. 1399-1406 ◽

Cited By ~ 18

Author(s):

Matthew S. Karafin ◽

Joshua J. Field ◽

Jerome L. Gottschall ◽

Gregory A. Denomme

Keyword(s):

Sickle Cell Disease ◽

Blood Cell ◽

Sickle Cell ◽

Red Blood Cell ◽

Adult Patients ◽

Cell Disease

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Caring for Patients with Sickle Cell Disease during a Pandemic: Continuing to Provide Automated Red Blood Cell Exchange Transfusions in Difficult Times

Blood ◽

10.1182/blood-2020-142535 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 25-26

Author(s):

Richard Curtis Godby ◽

Ashton Kornbrust ◽

Denis Noubouossie ◽

Jose Lima ◽

Marisa B. Marques ◽

...

Keyword(s):

Sickle Cell Disease ◽

Blood Cell ◽

Sickle Cell ◽

Red Blood Cell ◽

Blood Product ◽

T Test ◽

World Health ◽

Cell Disease ◽

Global Pandemic ◽

The Mean

Introduction: The World Health Organization declared COVID-19 a global pandemic on 03/11/20. Subsequent concerns around caring for patients with sickle cell disease who require automated red blood cell (RBC) exchange transfusions emerged, especially in the setting of physical distancing and national shortages in blood product supplies. In this vulnerable population at high risk of allo-immunization, ideal transfusion parameters (e.g., antigen optimization) will likely grow increasingly difficult to satisfy and require careful evaluation and strategic planning. Methods: Automated RBC exchange transfusions were performed at the University of Alabama at Birmingham (UAB) in patients with sickle cell disease for a variety of clinical indications with the primary objective of lowering the amount of Hemoglobin S (goal 15%) and replacing it with Hemoglobin A. We collected the number of weekly RBC exchange transfusions performed and then compared the frequencies between 01/05/20 and 03/14/20 (pre-pandemic) to those between 03/15/20 and 08/01/20 (intra-pandemic) using a one-tailed t-test. We also examined the number of RBC units ordered per week at UAB, in both the inpatient and outpatient settings, shortly before and after the declaration of a global pandemic using a one-tailed t-test. Results: The mean frequency of RBC exchange transfusions performed per week was 8.1 [standard deviation 2.3] pre-pandemic and 8.6 [2.3] intra-pandemic (Figure 1a). There was no statistically significant difference (p=0.27) in the frequency between these two periods. Shortly prior to the start of the pandemic (02/23/20-03/14/20), a mean of 77.3 [17.9] units/week were ordered for outpatient RBC exchange transfusions. Shortly after the start of the pandemic (03/15/20-04/26/20), a mean of 55.3 [22.8] units/week were ordered for outpatient RBC exchange transfusions, which was also not significantly different (p=0.09). During this time period, the mean number of RBC units per week ordered in the inpatient surgical setting significantly declined from 719.3 [43.1] to 390.0 [46.8] as elective procedures were delayed (p<0.005) (Figure 1b). Conclusions/Future Directions: The frequency of automated RBC exchange transfusions performed at UAB did not decrease after the onset of the pandemic. UAB was able to continue caring for patients with sickle cell disease receiving RBC exchange transfusions as the pandemic emerged and national blood product supplies declined despite a similar overall demand. Interestingly, there was also a concomitant decrease in the demand for RBCs from inpatient surgical settings as elective procedures were delayed, possibly contributing to the blood bank's ability to maintain ideal transfusion parameters and perform antigen optimization of transfused RBCs. As the COVID-19 pandemic continues, the national shortage of blood product supplies will likely worsen and necessitate multidisciplinary efforts, including intra-institutional and inter-institutional collaborations, to continue caring for patients with sickle cell disease receiving RBC exchange transfusions. Furthermore, community education, safely structured blood drives, and other efforts to encourage donations are essential to maintain the national blood product supply. Disclosures No relevant conflicts of interest to declare.

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End-Alveolar Carbon Monoxide As a Measure of Erythrocyte Survival and Hemolytic Severity in Sickle Cell Disease

Blood ◽

10.1182/blood.v124.21.2696.2696 ◽

2014 ◽

Vol 124 (21) ◽

pp. 2696-2696

Author(s):

Caterina P. Minniti ◽

John H. Baird ◽

Dihua Xu ◽

Laurel Mendelsohn ◽

Rehan Saiyed ◽

...

Keyword(s):

Sickle Cell Disease ◽

Blood Cell ◽

Life Span ◽

Sickle Cell ◽

Red Blood Cell ◽

Adult Patients ◽

Red Cell ◽

Cell Disease ◽

Indirect Bilirubin ◽

Cell Lifespan

Abstract Introduction: In sickle cell disease, polymerization of sickle hemoglobin gives rise to changes in the integrity and viability of the erythrocyte, leading to both extravascular and intravascular hemolysis and shortened life-span. Current gold standard techniques to estimate RBC survival require that a label be placed on the cells that can be followed while the RBCs age in the circulation. These studies are infrequently employed in practice, due to the need for multiple blood draws and an extended period of observation (6 weeks) in order to obtain the necessary time points. Endogenous CO is a technique that has been used in multiple studies to assess RBC survival, based upon the principal that virtually all CO produced in human beings results from cleavage of the α-methene bond of heme and is completely excreted via the lungs. Because RBC destruction accounts for approximately 80% of heme turnover in the body, endogenous CO production can be used as a quantitative indicator of RBC life span. Furne has reported on the development of a simple, rapid, and noninvasive method for determining RBC life span based on gas chromatography measurement of exhaled alveolar CO (EACO) concentration immediately upon awakening corrected for atmospheric CO, as determined with a device that simulates the body's equilibration with CO. We set up to investigate the use of early morning end-alveolar CO (EACO) concentration as a quantitative measure of RBC life span and correlate it to indirect measurements of hemolytic rate in subjects with sickle cell disease. Material and methods: EACO was measured within 24 hours of breath collection in 67 SCD adult patients (67 HbSS, 4 HbSC and 1 HbSb-Thalassemia) and 14 HbAA race matched controls on an Ametrek TA 7000 Gas Purity Monitor. Subjects were at steady state, at least 30 days from transfusion or acute exacerbations. Breath samples were collected immediately upon awakening the same day as blood sampling for additional tests. EACO groups were compared using the Mann-Whitney test. EACO values were used to calculate the red blood cell lifespan by the method of Furne (PMID 12878986), using the calculation: RBC survival (days) = 1,380 · [Hb]/EACO. Correlation analyses are reported using Pearson's correlation coefficient. The statistical significance was set at a two-side p < .05. Analyses were performed with R version 2.13.1 (2011-07-08). Results: EACO was nearly four-fold higher in patients than controls (median 2.25 vs. 0.58 ppm, p<.0001, figure). In patients with SCD, unadjusted EACO values were correlated with %HbS (p<.01), indirect markers of hemolysis: absolute reticulocyte count (p<.02), and percentage of reticulocytes (p<.01), indirect bilirubin (p<.001), and negatively with plasma nitrite (p<.02). EACO-derived red blood cell lifespan correlated negatively with LDH (p<.04), hematocrit (p<.01), hemoglobin (p<.06) indirect bilirubin (p<.01), absolute and percent reticulocyte counts (p<.01), AST (p<.01), and %HbS (p<.01). Conclusions: Our results confirm the usefulness of exhaled CO measurement as a marker of hemolysis and red cell survival and in substantial, prospective cohort of adult patients with SCD, confirming and considerably extending results of other investigators. EACO-derived RBC survival can be used to update estimates from Crosby in 1955 regarding the proportion of overall hemolysis falling into the extravascular and intravascular categories, the latter related to oxidative stress and scavenging of nitric oxide. This assay may be a useful biomarker in clinical trials of therapeutic interventions aimed at improving red cell life-span in SCD and other hemolytic anemias. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

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