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CASE SERIES  
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Automated red cell exchange in sickle cell disease patients: Retrospective analysis of series of cases in a tertiary care hospital in Eastern India


1 Department of Transfusion Medicine, IMS and SUM Hospital, Siksha O Anusandhan (Deemed to be University), Bhubaneswar, Odisha, India
2 Department of Clinical Hematology, IMS and SUM Hospital, Siksha O Anusandhan (Deemed to be University), Bhubaneswar, Odisha, India

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Date of Submission25-Nov-2021
Date of Decision19-Jul-2022
Date of Acceptance31-Jul-2022
Date of Web Publication12-Dec-2022
 

   Abstract 


Red cell exchange is important to treat acutely ill sickle cell patients, but it is time-consuming. An automated red cell exchange technique using cell separators developed by different manufacturers helps in removal of sickled hemoglobin and improving blood viscosity. The use of these cell separators permits automated red cell exchange to be performed safely and smoothly with the isovolemic hemodilution. The retrospective analysis of seven cases for automated red cell exchange was performed in IMS and SUM Hospital from September, 2019 to July 2021. One procedure was performed on COBE Spectra Apheresis System and the rest six using Spectra Optia Apheresis System. These procedures were performed on the patients and the decision to perform these procedures was based on clinical indications for red cell exchange following the ASFA guidelines 2019. After only one procedure, all the patients have their sickle hemoglobin reduced to a safe level. End hematocrit was observed to be 33% in three cases, 31.9% in one case, and 30% in the rest three. A total number of red blood cells (RBCs) transfusion in all the cases were 7, 6, 8, 5, 6, 7, and 7 with an average hematocrit of 55%, 56%, 54%, 56%, 55%, 51.7%, and 52.2%, respectively. All the patients partially phenotypically matched, leukoreduced, gamma-irradiated, fresh packed RBCs were provided. Automated red cell exchange came out to be successful in reducing the symptoms along with the improvement in laboratory parameters. New-generation automated apheresis equipment like Spectra Optia, the case series provides better monitoring and also reduces apheresis-related complications.

Keywords: Apheresis, automated red cell exchange, COBE spectra, sickle cell disease, Spectra Optia


How to cite this URL:
Kanungo G, Nagrath P, Samal P, Mishra S. Automated red cell exchange in sickle cell disease patients: Retrospective analysis of series of cases in a tertiary care hospital in Eastern India. Asian J Transfus Sci [Epub ahead of print] [cited 2023 Jan 28]. Available from: https://www.ajts.org/preprintarticle.asp?id=363218



   Introduction Top


India has a significant burden of hemoglobinopathies, varying in different regions of the country, majorly sickle cell disease (SCD) with a prevalence of 0%–18% in Northeastern India, 0%–33.5% in Western India, 22.5%–44.4% in Central India, and 1%–40% in Southern India.[1],[2] For many years, blood transfusion has always been the mainstay of treatment for sickle cell anemia and in maintaining hemoglobin (Hb) threshold of 9–9.5 g/dl.[3] However, repeated transfusions put the patient at risk of alloimmunization, iron overload, and risk of infectious diseases. Alloimmunization is one of the most common complications of multiple transfusions in SCD patients, and its incidence varies from 2% to 47% in various studies.[4] Apart from these, vaso-occlusive events, recurrent pain crisis, and avascular necrosis of bone are other complications. Red cell exchange is important in the treatment of these complications and provides immediate relief by reducing sickle hemoglobin (HbS) concentration and blood viscosity of the patient. Red blood cell (RBC) exchange is also beneficial in the treatment of fewer postoperative complications like acute chest syndrome.[5]


   Case Series Top


The case series was performed in the Department of Transfusion Medicine of the Institute of Medical Sciences and SUM Hospital, Bhubaneswar, between September 2019 and October 2021.

Type and screen

Blood typing and antibody screening with extended Rh typing with Kell was performed on an automated platform Galileo (Immucor, USA) using solid-phase red-cell adherence (SPRCA). Antibody screening was also done by three-cell panel (Bio-Rad, GmbH, Switzerland) by the column agglutination technology. Eleven-cell panel (Bio-Rad, GmbH, Switzerland,) was used for the identification of alloantibodies.

Two cases were detected to be alloimmunized with a single alloantibody.

Red blood cell units

All units were ABO RhD typed and extended Rh type with Kell typed, fresh, leukodepleted, antigen negative, and antihuman globulin (AHG) compatible RBC units from the inventory for possible RBC exchange.

Automated red blood cell exchange

The decision to perform this procedure on SCD patients was based on clinical indications for red cell exchange following the ASFA guidelines 2019. One procedure was performed on COBE Spectra (Terumo BCT, Lakewood, Colorado, USA) and the rest eight were performed using Spectra Optia (Terumo BCT, Lakewood, Colorado, USA) cell separator. Informed consent was obtained from all the patients after consultation with a clinical hematologist and transfusion medicine consultant and the procedure was performed.

Double-lumen central catheter into the internal jugular or femoral veins was inserted. There were no procedure-related complications.

Medical records were retrospectively reviewed for demographic and clinical data, laboratory data, and radiological data to collect the following information: age, gender, symptoms, disease duration before RBCx, treatment regimens, complications, degrees of improvement, and outcome.

Case details

Case details are shown in [Table 1]
Table 1: Clinical features, procedure parameters of red blood cell exchange (n=9)

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.

Management and outcome

A total of nine patients with SCD were admitted to our hospital and automated red cell exchange was performed using a cell separator. Out of these, one was female (11%) and the rest eight (89%) were male, as shown in [Table 1].

A total of 1 (11.11%) patient was in the age group of 11–20 years, 6 (66.67%) were in the age group of 21–30 years, and 2 (22.22%) were 31–40 years, as depicted in [Figure 1]. None of the patients was of age <10 or more than 41 years, as shown in [Table 1].
Figure 1: Graphical representation of Age distribution of Patients

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Out of nine patients, 2 (22.2%) had blood Group A +ve, 3 (33.33%) had blood group B +ve, 3 (33.33%) were of blood group O +ve, and 1 (11.1%) was AB +ve, as shown in [Table 1].

The details of the procedure performed on all the nine patients are explained in [Table 1] showing total blood volume, inlet blood volume processed, type of cell separator used, number of RBCs used, and average hematocrit of the RBCs used.

Anticoagulant

In all the procedures, acid citrate dextrose (ACD) was used as an anticoagulant with volumes ranging from 176 to 319 ml. The amount of ACD depends on the volume of blood processed and what volume exchanged we have taken that procedure for. A range of 57–273 ml of ACD was gone to the patient by end of the procedure.

Preprocedural investigation

Preprocedural investigation included complete blood count, type and screen, Hb electrophoresis, and serum electrolytes such as ionized calcium, magnesium, sodium, potassium, serum bilirubin, and electrocardiogram. Some of which are written below in [Table 2].
Table 2: Pre- and post-procedural blood investigation results of all nine cases

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Hemoglobin electrophoresis

After completion of only one cycle for all the cases, HbS level reduced to a level: from 68.5% to 26% for Case 1, from 70% to 40% for Case 2, from 69% to 25% for Case 3, from 70% to 31% for Case 4, from 63.4% to 27% for Case 5, from 70.1% to 31.7% for Case 6, from 69% to 32% for Case 7, from 67.8% to 32% for Case 8, and from 58.7% to 32% for Case 9 as clearly depicted in [Figure 2].
Figure 2: Case-wise comparison of pre- and post-HbS. HbS = Sickle hemoglobin

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Blood transfusions

A total number of packed RBCs transfused in all the cases were 7, 6, 8, 5, 6, 7, 7, 7, and 6 with an average hematocrit of 63%, 64%, 61%, 65%, 62%, 60%, 61%, 67%, and 64%, respectively.


   Discussion Top


Transfusions in SCD patients alleviate anemia, reduce the blood viscosity and prevent complications. Simple transfusion is an easy procedure and is required in acute phases, in which there is a rapid need to increase oxygen-carrying capacity.[6] Chronic transfusion limit increment in oxygen carrying capacity as well as keep up a lower level of HbS. Yet simple transfusion can cause volume overburden, increased blood viscosity and iron deposition. To overcome the limitations of simple transfusion, red cell exchange transfusion is the technique, in which the patient red cells are removed either manually or by automated apheresis equipment and replaced with donor (allogenic) red cells. Red cell exchange (RCE) is superior to simple transfusion in the treatment of patients with SCD or respiratory failure or multiorgan failure. The observational study by Hulbert et al. suggested that RCE therapy is better than simple transfusion in suspected stroke and a study by Hassell KL also proves it to be superior in patients of multiorgan failure.[7],[8] The guidelines recommend that one volume red cell volume exchange (RCE) transfusion can replace 65% of the patient's original RBCs with a fraction of cells remaining of 35%.[9]

The British Society for Haematology guidelines also underlines the fact that a large referral center managing patients with SCD should have facilities and trained staff for automated exchange transfusion and should be able to provide red cells that are negative for corresponding antibodies if any clinically significant antibody is present.[10]

Studies suggest that SCD patients benefit from prophylactic extended antigen matching and especially it is significant in patients who have already made alloantibodies or patients with warm reacting autoantibodies.[11]

The choice for automated red cell exchange relies on the accessibility of apheresis services, adequate intravenous access, preferably leukoreduced, Rh- and Kell-matched, and AHG crossmatch compatible blood products. Red cell exchange allows continuous monitoring of the patient in addition to patient comfort and safety. Red cell exchange is a relatively safe procedure still the patient is at risk for transfusion-associated adverse events including apheresis risks. The most commonly encountered include risk of central venous catheter thrombosis and hemorrhage.[11],[12]

Automated Red Cell Exchange (RCE) performed in these patients came out to be successful in reducing the symptoms along with the improvement in laboratory parameters. In our study, all nine patients who underwent automated red cell exchange tolerated the procedure well. Out of nine cases, RCE was done for three cases for preoperative for general anesthesia, one of which patient admitted for total hip replacement surgery, two patients for lap cholecystectomy, two patients for the vaso-occlusive crisis, and two patients for sickle cell hepatopathy in new-generation automated apheresis equipment like Spectra Optia, providing better monitoring of the procedure and also reduced apheresis-related complications with an average postprocedure HbS of 30.74%.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Hockham C, Bhatt S, Colah R, Mukherjee MB, Penman BS, Gupta S, et al. The spatial epidemiology of sickle-cell anaemia in India. Sci Rep 2018;8:17685.  Back to cited text no. 1
    
2.
Serjeant GR, Ghosh K, Patel J. Sickle cell disease in India: A perspective. Indian J Med Res 2016;143:21-4.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Liumbruno G, Bennardello F, Lattanzio A, Piccoli P, Rossetti G. Recommendations for the transfusion of red blood cells. Blood Transfus 2009;7:49-64.  Back to cited text no. 3
    
4.
Campbell-Lee SA, Kittles RA. Red blood cell alloimmunization in sickle cell disease: Listen to your ancestors. Transfus Med Hemother 2014;41:431-5.  Back to cited text no. 4
    
5.
Swerdlow PS. Red cell exchange in sickle cell disease. In: Berliner N, Lee S, Linenberger M, Vogelsang M, Bajus J, editors. Hematology: American Society of Hematology Education Program Book. Washington, DC: American Society of Hematology; 2006. p. 48.  Back to cited text no. 5
    
6.
Swerdlow PS. Red cell exchange in sickle cell disease. Hematology Am Soc Hematol Educ Program 2006;2006:48-53.  Back to cited text no. 6
    
7.
Hulbert ML, Scothorn DJ, Panepinto JA, Scott JP, Buchanan GR, Sarnaik S, et al. Exchange blood transfusion compared with simple transfusion for first overt stroke is associated with a lower risk of subsequent stroke: A retrospective cohort study of 137 children with sickle cell anemia. J Pediatr 2006;149:710-2.  Back to cited text no. 7
    
8.
Hassell KL, Eckman JR, Lane PA. Acute multiorgan failure syndrome: A potentially catastrophic complication of severe sickle cell pain episodes. Am J Med 1994;96:155-62.  Back to cited text no. 8
    
9.
Guru PK, O Horo JC, Lehrke HD, Winters JL, Wilson JW. Exchange transfusion for babesiosis when, how, and how long? Indian J Crit Care Med 2016;20:674-6.  Back to cited text no. 9
[PUBMED]  [Full text]  
10.
Davis BA, Allard S, Qureshi A, Porter JB, Pancham S, Win N, et al. Guidelines on red cell transfusion in sickle cell disease. Part I: Principles and laboratory aspects. Br J Haematol 2017;176:179-91.  Back to cited text no. 10
    
11.
Baron JM, Baron BW. Red cell exchange is not effective for patients with sickle cell anaemia and coexisting warm autoantibody haemolysis. Blood Transfus 2010;8:303-6.  Back to cited text no. 11
    
12.
Yeral M, Boga C, Oguzkurt L, Asma S, Kasar M, Kozanoglu I. Short-term central venous catheter complications in patients with sickle cell disease who undergo apheresis. J Thromb Thrombolysis 2014;37:97-101.  Back to cited text no. 12
    

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Correspondence Address:
Girijanandini Kanungo,
Department of Transfusion Medicine, IMS and SUM Hospital, Siksha O Anusandhan (Deemed to be University), Bhubaneswar - 751 003, Odisha
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ajts.ajts_174_21



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