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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 4  |  Issue : 2  |  Page : 237-239

Resolution by adsorption-elution method and transfusion support to a patient of thalassemia intermedia with anti-c alloantibody


1 Department of Transfusion Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
2 Department of Medical Oncology and Hematology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India

Date of Submission17-May-2019
Date of Acceptance17-Sep-2019
Date of Web Publication17-Oct-2019

Correspondence Address:
Dr. Somnath Mukherjee
Department of Transfusion Medicine, All India Institute of Medical Sciences, Bhubaneswar
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/GJTM.GJTM_34_19

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  Abstract 


Transfusion support remains the mainstay of supportive treatment for thalassemia patients in spite of recent advances such as stem cell transplantation and gene therapy. Thalassemia intermedia (TI) patients generally do not require regular transfusion support but can develop severe anemia, requiring frequent transfusion during the period of stress such as infection and pregnancy. The genetic disparity of red blood cell antigens between donor and recipient leads to sensitization and resulting development of alloantibodies. Red cell alloimmunization leads to incompatiblity and delays in issue of compatible units. Majority of these alloantibodies were against Rh and Kell blood group. Here we report a case of 28 years old TI who developed anti-c following multiple transfusions since the age of 18 years. The patient was transfused with c antigen-negative, A positive anti-human globulin cross-matched blood unit. Thus, we emphasize to include antibody screening routinely in multi-transfused patients and provision of at least Rh phenotype-matched blood to prevent further alloimmunization.

Keywords: Antibody screening, anti-c alloantibody, thalassemia intermedia


How to cite this article:
Mishra D, Dash PK, Prakash S, Mukherjee S. Resolution by adsorption-elution method and transfusion support to a patient of thalassemia intermedia with anti-c alloantibody. Glob J Transfus Med 2019;4:237-9

How to cite this URL:
Mishra D, Dash PK, Prakash S, Mukherjee S. Resolution by adsorption-elution method and transfusion support to a patient of thalassemia intermedia with anti-c alloantibody. Glob J Transfus Med [serial online] 2019 [cited 2019 Nov 12];4:237-9. Available from: http://www.gjtmonline.com/text.asp?2019/4/2/237/269385




  Introduction Top


The clinical phenotype of thalassemia ranges between severe transfusion-dependent thalassemia major and the asymptomatic carrier state.[1] The term “thalassemia intermedia (TI)” is used to describe individuals with a less severe phenotype than thalassemia major; these individuals tend to have anemia of nonsevere type; hence, they are not transfusion dependent during childhood. The typical age of presentation is 2–4 years of age.[2] These patients also are more prone to develop alloantibody than thalassemia major patients. Mostly, the alloantibodies are developed against Rh antigens. Alloantibody against Rh antigens are mainly of immunoglobulin (IgG) type and causes delayed hemolytic transfusion reaction (DHTR). Here, we report a case of TI patient with anti-c alloantibody causing cross-match incompatibility which leads to increased turnaround time in issuing of compatible units. Hence, we emphasize the importance of antibody screening in multi-transfused patients and to provide phenotype-matched blood units to prevent further alloimmunization and untoward reaction.


  Case Report Top


A 28-year-old nonpregnant female patient with severe anemia and symptoms of breathlessness, weakness required transfusion. She was diagnosed as a case of TI at the age of 12 years, but the age of first transfusion was 18 years, and the last transfusion was 5 months back. Her laboratory features were as follows: hemoglobin (Hb) of 4.6 g/dl, hematocrit 18.3%, mean corpuscular volume 60.4 FL, mean corpuscular Hb concentration 25.4 g/dl, and indirect bilirubin 7 mg/dl. Three units of packed red blood cells (PRBC) transfusion request was received by transfusion laboratory. ABO and Rh D typing was done with test tube technique. Cell grouping was done using commercially available Anti-A, Anti-B, Anti-D (Tulip Diagnostic, Goa, India) and serum grouping by in-house prepared pooled A cell, B cell, and O cell, respectively. Her ABO and Rh D typing was A Rh D positive. Cross-matching was done on anti-human globulin (AHG, IgG + C3d) gel card (Tulip Diagnostic, Goa, India). However, the cross-match was incompatible (2+). The blood sample was screened for presence of irregular antibodies using a commercial antibody screening panel (ID-DiaCell I-II-III Asia, BIO-RAD).

The screening test was positive with 3-cell panel, in cell II (4+) and cell III (4+) with negative autocontrol and negative direct anti-globulin test as shown in [Table 1]. The antibody identification using 11 cell panels (ID-DiaCell Asia, BIO-RAD) had shown the possibilities of antibodies were anti-c, anti-E, anti-S, and anti-Lua [Table 2]. Extended Rh phenotype was done using antisera (Tulip Diagnostic, Goa, India) by test-tube technique. Patient's Rh phenotype was C +c−E−e+ and also positive for S, Lua antigens. Hence, anti-S and anti-Lua antibodies were ruled out. Anti-E antibody was ruled out by adsorption-elution method. Adsorption and elution were done with ZZAP (dithiothreitol + cysteine activated papain) reagent, Elu-kit (BIO-RAD), respectively. First c antigen was absorbed by incubating patient's serum with ZZAP treated c + E− red cell as described by AABB technical manual.[3] After complete adsorption, when adsorbed serum was tested with 11-cell panel, no reaction was observed suggests no anti-E was present [Figure 1]. Elute from this c + E− adsorbed cells were obtained and again tested for antibody identification with 11 cell panel and the reaction pattern confirmed that the antibody was anti-c [Figure 2].
Table 1: Antibody screening test and DCT

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Table 2: Antibody identification panel

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Figure 1: Antibody identification with adsorbed serum after complete adsorption with c + E- red cell

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Figure 2: Antibody identification from the elute of adsorbed cells (c + E-) to confirm anti-c

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Three units of A positive, c and E antigen-negative, leukocyte reduced PRBC was transfused to prevent the formation of anti-E. The patient was discharged in stable condition with Hb 8.2 g/dl. After 4 months, she was again admitted with Hb 4.8 g/dl but this time antibody screening was negative. She was now also transfused with three units of A positive, c and E antigen-negative, leukocyte reduced PRBC, and she tolerated the red cell product without any untoward event.


  Discussion Top


Some TI patients remain asymptomatic until they attain adulthood and may present with moderate anemia which do not require regular transfusions, whereas others become symptomatic from as early as 2 years of age.[4],[5] Transfusion becomes necessary in TI patients with severe anemia (Hb below 7 g/dl) due to ineffective erythropoiesis which leads to chronic hypoxia sometimes during infection-induced aplastic crisis and heart disease.[6]

Anti-c is clinically significant IgG type Rh antibody and associated with hemolytic disease of new-born, and DHTR as a single or with anti-E antibody.[7] A study by Thakral et al. showed the RBC alloimmunization incidence in transfused patients was 3.4%, and most common antibody specificity was anti-c (38.8%).[8] Sachan et al. also described the role of antibody screening in pretransfusion testing and provision of at least Rh, Kell phenotype-matched blood in repeated transfused patients.[9]

Anti-c was identified in our case during pretransfusion testing in AHG phase, suggested IgG antibody against c-antigen from transfused PRBC as the likely cause. Her Rh phenotype had shown the presence of C, e and absence of c, E antigen, and adsorption-elution study supported presence of Anti-c alloantibody in patient serum. The patient was transfused with cross-matched and compatible c, E antigen-negative blood units in AHG phase with column agglutination method. On follow up after 4 months, she was again required transfusion with Hb% 4.8. Previously identified anti-c or any other additional alloantibodies were not detected. This time she was again transfused c and E antigen-negative PRBC, and no adverse event was reported. Hence, we strongly recommend antibody screening, and at least extended Rh phenotype-matched blood transfusion for multi-transfused thalassemia patients.

In majority of peripheral centers of India before transfusion only ABO, Rh D blood grouping, and immediate spin cross-matching (detect ABO incompatibility) are done to provide blood transfusion. Most of the antibodies other than ABO often arise after sensitization to foreign red cell antigen through pregnancy, transfusion. In most of peripheral centers, before transfusion testing does not include antibody screening and identification, which is essential to detect clinically significant antibodies against minor blood group antigens such as Rh, Kell, Kidd, and Duffy to provide safe blood. Patients of sickle cell disease and thalassemia on continuous transfusion support may be considered for transfusion of phenotypically matched RBC units to prevent alloimmunization.[10]


  Conclusion Top


Possibility of alloimmunization is high in multi-transfused patients such as thalassemia. Thus, we suggest to include the antibody screening, identification in pretransfusion testing especially for multi-transfused patients such as thalassemia and to provide at least extended Rh-matched PRBC transfusion.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Rund D, Rachmilewitz E. Beta-thalassemia. N Engl J Med 2005;353:1135-46.  Back to cited text no. 1
    
2.
Rachmilewitz EA, Giardina PJ. How I treat thalassemia. Blood 2011;118:3479-88.  Back to cited text no. 2
    
3.
Roback JD, Grossman BJ, Harris T, Hiller CD, editors. Technical Manual – American Association of Blood Banks. 17th ed. Bethesda: AABB Press; 2011. p. 925-6.  Back to cited text no. 3
    
4.
Taher A, Isma'eel H, Cappellini MD. Thalassemia intermedia: Revisited. Blood Cells Mol Dis 2006;37:12-20.  Back to cited text no. 4
    
5.
Cappellini MD, Musallam KM, Taher AT. Insight onto the pathophysiology and clinical complications of thalassemia intermedia. Hemoglobin 2009;33 Suppl 1:S145-59.  Back to cited text no. 5
    
6.
Shawky RM, Kamal TM. Thalassemia intermedia: An overview. Egypt J Med Hum Genet 2012;13:245-55.  Back to cited text no. 6
    
7.
Hillman NM. Fatal delayed hemolytic transfusion reaction due to anti-c+E. Transfusion 1979;19:548-51.  Back to cited text no. 7
    
8.
Thakral B, Saluja K, Sharma RR, Marwaha N. Red cell alloimmunization in a transfused patient population: A study from a tertiary care hospital in North India. Hematology 2008;13:313-8.  Back to cited text no. 8
    
9.
Sachan D, Jayakumar R, Varghese J, Rela M. An acute hemolytic transfusion reaction due to the “anti-c” rhesus antibody: A case report emphasizing the role of transfusion medicine. Asian J Transfus Sci 2015;9:213-5.  Back to cited text no. 9
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10.
Afenyi-Annan A, Brecher ME. Pre-transfusion phenotype matching for sickle cell disease patients. Transfusion 2004;44:619-20.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2]



 

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