A Comparison of Chronic Manual and Automated Red Blood Cell Exchange Transfusion in Sickle Cell Disease Patients From Two Comprehensive Care Centres in the United Kingdom

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3430-3430 ◽  
Author(s):  
Kevin H.M. Kuo ◽  
Richard Ward ◽  
Jo Howard ◽  
Paul Telfer

Abstract Abstract 3430 Chronic transfusion is indicated in the treatment and prevention of sickle cell disease (SCD) related-complications. Red blood cell exchange (RBCX) is preferred over top-up transfusion as it enables iron balance and rapid reduction of sickle hemoglobin (HbS) without increasing blood viscosity. RBCX can be done manually by sequential phlebotomy and transfusion or by automated apheresis, but the two methods have not been formally compared. Bart's Health NHS Trust (BHT) performs manual RBCX and Guy's and St. Thomas NHS Trust (GSTT) automated RBCX. Manual RBCX consists of isovolumetric exchange 15 to 20 mL/kg of venesected blood for normal saline and packed RBC, generally at a 1:1 ratio. Automated RBCX utilizes the Spectra Optia Apheresis System with a fractional cell remaining between 24 and 44, end Hct within 0.02 of pre-RBCX Hct, and a fluid replacement balance of 100%. The indications for RBCX and pre-RBCX HbS or hemoglobin SC (HbSC) target (30% or 50%, depending on indication) are the same in both units. All packed RBC were leukodepleted, HbS negative, ≤ 7 days old, and phenotypically matched for C, D, E, and Kell. A retrospective observational cohort study as part of a quality assurance audit was conducted examining all SCD patients attending regular RBCX at BHT and GSTT between 1 May 2011 and 30 April 2012. Fifty-one patients (21 manual, 30 automated), totaling 401 RBCX sessions were included. Ten patients in the manual group and 7 patients in the automated group targeted an HbS of < 30%, while the rest were targeted to < 50%. There was no significant difference in baseline characteristics between the two groups. Peripheral vein was the most common method of cannulation in manual RBCX while temporary central venous catheterization was most common in automated RBCX (P < 0.0001). Time interval differed between manual and automated RBCX (median 4.4 vs. 7.1 weeks, P < 0.0001). Both groups consistently achieved a post-RBCX Hct < 0.350. More patients on automated RBCX were able to consistently achieve their prescribed HbS or HbSC target (defined as > 2/3 of RBCX sessions) than on manual RBCX (37% vs. 10%, unadjusted OR 5.5, 95% CI 1.07 – 28.22, P = 0.048). When adjusted for the prescribed pre-RBCX HbS or HbSC target, the Mantel-Haenszel OR estimate was 4.72 (95% CI 0.885 – 25.17). Older age was also associated with consistently achieving the prescribed HbS or HbSC target (P = 0.010 on multivariable logistric regression). Although the yearly volume of RBC and donor units used were significantly higher in the automated group (241.1 mL/kg/year and 55 units/year) compared to the manual group (127 mL/kg/year and 32 units/year), there was no difference in the rate of allo-antibody formation. There was no difference in the mean ferritin trend between manual and automated RBCX in non-chelated patients (−0.068 ± 1.439 vs. −0.297 ± 2.027 ug/L/day, P = 0.439). Automated RBCX was better at maintaining a near zero iron balance than manual RBCX due to considerable individual variability in the manual group (kurtosis statistic 13.221 ± 0.935 SE vs. 0.398 ± 1.121 SE). It took a significantly longer time to perform a manual RBCX session than an automated one (257 min. vs. 115 min.). There was no significant difference in the number of adverse events. The number of RBCX sessions that had to be converted to top-up transfusions in the manual group because of low pre-RBCX haematocrit far exceeded that of the automated group (11 vs. 0 sessions). None of the patients in either group had new or progressive neurological events. Manual RBCX, despite an optimized protocol and strict adherence to treatement, is inferior to automated RBCX in being able to consistently achieve the prescribed HbS target, although consistent adherence with automated RBCX can also be challenging. Automated RBCX requires less time to perform than manual RBCX, but involves higher RBC use and more donor exposure. Adverse events and antibody formation are the same for both methods. Automated RBCX can maintain a near zero iron balance in most patients without the use of iron chelation and with less individual variability than manual RBCX. The results suggest that automated RBCX is the preferred RBC exchange method in controlling HbS in SCD patients. Further longitudinal study is required to determine whether a better control of HbS translates into differences in patient-related outcomes. Disclosures: No relevant conflicts of interest to declare.

2015 ◽  
Vol 22 (3) ◽  
pp. 34-40
Author(s):  
Abdulrahman S. Alboog ◽  
Taher M. Tayeb ◽  
Mohammed O. Alsager ◽  
Salwa A. AlNajjar ◽  
Ghazi A. Damanhouri ◽  
...  

The treatment of patients with sickle cell disease frequently requires transfusion of red blood cells. Complications due to alloimmunization of red blood cells antigen remain a major risk as a post transfusion effect. The objective of this study is to determine the frequency of red cell alloimmunization in Jeddah, Saudi Arabia. A retrospective cross-section study of sickle cell disease patients at King Abdulaziz University Hospital between 2012 and 2013 was performed. Demographic characteristics and transfusion history was recorded. Blood samples were analyzed for alloimmunization using immunohematological technique. A total of 234 sickle cell patients were analyzed, of which 30 (12.8%) showed alloantibodies. A total of 43 alloantibodies were found out of which 28 belonged to Rh group, eight belonged to Kell while three belonged to MNS group. Demographic and transfusion characteristics were analyzed between alloimmunized and nonalloimmunized sickle cell disease patients. The rate of alloimmunization in Jeddah, Saudi Arabia was 12.8%. There was significant difference observed between alloantibodies detection between transfused patients compared to non-transfused patients. The consequences of red blood cell alloimmunization are highly significant and therefore immune hematological testing is highly recommended.  


Cells ◽  
2021 ◽  
Vol 10 (4) ◽  
pp. 811
Author(s):  
Camille Boisson ◽  
Minke A. E. Rab ◽  
Elie Nader ◽  
Céline Renoux ◽  
Celeste Kanne ◽  
...  

(1) Background: The aim of the present study was to compare oxygen gradient ektacytometry parameters between sickle cell patients of different genotypes (SS, SC, and S/β+) or under different treatments (hydroxyurea or chronic red blood cell exchange). (2) Methods: Oxygen gradient ektacytometry was performed in 167 adults and children at steady state. In addition, five SS patients had oxygenscan measurements at steady state and during an acute complication requiring hospitalization. (3) Results: Red blood cell (RBC) deformability upon deoxygenation (EImin) and in normoxia (EImax) was increased, and the susceptibility of RBC to sickle upon deoxygenation was decreased in SC patients when compared to untreated SS patients older than 5 years old. SS patients under chronic red blood cell exchange had higher EImin and EImax and lower susceptibility of RBC to sickle upon deoxygenation compared to untreated SS patients, SS patients younger than 5 years old, and hydroxyurea-treated SS and SC patients. The susceptibility of RBC to sickle upon deoxygenation was increased in the five SS patients during acute complication compared to steady state, although the difference between steady state and acute complication was variable from one patient to another. (4) Conclusions: The present study demonstrates that oxygen gradient ektacytometry parameters are affected by sickle cell disease (SCD) genotype and treatment.


Lab on a Chip ◽  
2021 ◽  
Author(s):  
Yuncheng Man ◽  
Debnath Maji ◽  
Ran An ◽  
Sanjay Ahuja ◽  
Jane A Little ◽  
...  

Alterations in the deformability of red blood cells (RBCs), occurring in hemolytic blood disorders such as sickle cell disease (SCD), contributes to vaso-occlusion and disease pathophysiology. However, there are few...


2001 ◽  
Vol 23 (3) ◽  
pp. 165-169 ◽  
Author(s):  
Tay S. Kennedy ◽  
Ellen B. Fung ◽  
Deborah A. Kawchak ◽  
Babette S. Zemel ◽  
Kwaku Ohene-Frempong ◽  
...  

Transfusion ◽  
2012 ◽  
Vol 53 (4) ◽  
pp. 704-709 ◽  
Author(s):  
Scott T. Miller ◽  
Hae-Young Kim ◽  
Debra L. Weiner ◽  
Carrie G. Wager ◽  
Dianne Gallagher ◽  
...  

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 27-28
Author(s):  
Kelly M. Knee ◽  
Amey Barakat ◽  
Lindsay Tomlinson ◽  
Lila Ramaiah ◽  
Zane Wenzel ◽  
...  

Sickle cell disease (SCD) is a severe genetic disorder caused by a mutation in hemoglobin (b6Glu-Val), which allows the mutant hemoglobin to assemble into long polymers when deoxygenated. Over time, these polymers build up and deform red blood cells, leading to hemolytic anemia, vaso-occlusion, and end organ damage. A number of recent therapies for SCD have focused on modulating the mutant hemoglobin directly, however, reduction or elimination of 2,3-DPG to reduce Hb S polymerization and RBC sickling has recently been proposed as a therapeutic strategy for SCD. Current clinical studies focus on activation of pyruvate kinase to reduce 2,3-DPG, however, direct targeting of the enzyme which produces 2,3-DPG; Bisphosphoglycerate Mutase (BPGM) may also be possible. In this study we evaluate the impact of elimination of 2,3-DPG on SCD pathology by complete knockout of BPGM in Townes model mice. Animals with complete knockout of BPGM (BPGM -/-) have no detectable 2,3-DPG, while animals that are heterozygous for BPGM (BPGM -/+) have 2,3-DPG levels comparable to Townes mice. Western Blot analysis confirms that BPGM -/- animals completely lack BPGM, while BPGM -/+ animals have BPGM levels that are nearly equivalent to Townes mice. As expected from the lack of 2,3-DPG, BPGM -/- animals have increased oxygen affinity, observed as a 39% decrease in p50 relative to Townes mice. Complete elimination of 2,3-DPG has significant effects on markers of hemolytic anemia in BPGM -/- mice. Mice lacking 2,3-DPG have a 60% increase in hemoglobin (3.7 g/dL), a 53% increase in red blood cell count, and a 29% increase in hematocrit relative to Townes mice. The BPGM -/- mice also have a 57% decrease in reticulocytes, and a 61% decrease in spleen weight relative to Townes animals, consistent with decreased extramedullary hematopoiesis. Consistent with the reduction in hemolysis, BPGM -/- animals had a 59% reduction in red blood cell sickling under robust hypoxic conditions. BPGM -/+ animals had hemoglobin, RBC, and hematocrit levels that were similar to Townes animals, and a similar degree of RBC sickling to Townes mice. Liver phenotype was similar across all variants, with areas of random necrosis observed in BPGM -/-, BPGM -/+ and Townes mice. Higher percentages of microcytic and/or hyperchromic RBCs were observed in BPGM -/- animals relative to BPGM -/+ or Townes animals. These results suggest that modulation of 2,3-DPG has a positive effect on RBC sickling and hemolytic anemia, which may have therapeutic benefits for SCD patients. However, the lack of improvement in organ damage suggests that modulation of 2,3-DPG alone may not be sufficient for complete elimination of SCD phenotypes, and further investigation of this therapeutic avenue may be necessary. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2012 ◽  
Vol 120 (3) ◽  
pp. 528-537 ◽  
Author(s):  
Karina Yazdanbakhsh ◽  
Russell E. Ware ◽  
France Noizat-Pirenne

Abstract Red blood cell transfusions have reduced morbidity and mortality for patients with sickle cell disease. Transfusions can lead to erythrocyte alloimmunization, however, with serious complications for the patient including life-threatening delayed hemolytic transfusion reactions and difficulty in finding compatible units, which can cause transfusion delays. In this review, we discuss the risk factors associated with alloimmunization with emphasis on possible mechanisms that can trigger delayed hemolytic transfusion reactions in sickle cell disease, and we describe the challenges in transfusion management of these patients, including opportunities and emerging approaches for minimizing this life-threatening complication.


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