|Year : 2019 | Volume
| Issue : 1 | Page : 101-104
Resolution of blood group discrepancy and transfusion support to a nontransfusion-dependent thalassemia patient with anti-C and anti-N alloantibodies
Debasish Mishra1, Rashmita Panigrahi2, Gopal Krushna Ray2, Dibyajyoti Sahoo1
1 Department of Transfusion Medicine and Blood Bank, All India Institute of Medical Science, Bhubaneswar, Odisha, India
2 Department of Transfusion Medicine, SCB Medical College, Cuttack, Odisha, India
|Date of Web Publication||22-Apr-2019|
Dr. Debasish Mishra
Department of Transfusion Medicine and Blood Bank, All India Institute of Medical Science, Bhubaneswar, Odisha
Source of Support: None, Conflict of Interest: None
Nontransfusion-dependent thalassemia (NTDT) patients generally do not require regular blood transfusion, but may be transfused before surgery. Blood group discrepancy exists when forward grouping and reverse grouping do not match. Pretransfusion testing includes ABO/Rh typing, antibody screening, and cross-matching. Forward grouping and reverse grouping were done using monoclonal anti-A, anti-B, and anti-D (Tulip Diagnostics, Goa, India) and in-house prepared 5% A, B, and O cell by conventional tube test method, respectively. Antibody screening and identification were done using 3-cell panel and 11-cell panel (Ortho Clinical Diagnostics, USA), respectively. In our case, we found a discrepancy in reverse grouping and incompatibility due to anti-c and anti-N alloantibodies. Three units of O positive, c and N antigen negative blood units were transfused to this patient. The patient was also managed by hematinics, antibiotic, and bed rest. Antiglobulin cross-matching must be done in all types of compatibility testing of RBC units. Rh and K phenotype-matched PRBC reduced the chances of alloimmunization. Proper coordination between the physician and specialist in transfusion medicine is required in case of alloantibody formation.
Keywords: Anti-c and anti-N, group discrepancy, nontransfusion-dependent thalassemia
|How to cite this article:|
Mishra D, Panigrahi R, Ray GK, Sahoo D. Resolution of blood group discrepancy and transfusion support to a nontransfusion-dependent thalassemia patient with anti-C and anti-N alloantibodies. Glob J Transfus Med 2019;4:101-4
|How to cite this URL:|
Mishra D, Panigrahi R, Ray GK, Sahoo D. Resolution of blood group discrepancy and transfusion support to a nontransfusion-dependent thalassemia patient with anti-C and anti-N alloantibodies. Glob J Transfus Med [serial online] 2019 [cited 2019 Jul 23];4:101-4. Available from: http://www.gjtmonline.com/text.asp?2019/4/1/101/256747
| Introduction|| |
Nontransfusion-dependent thalassemia (NTDT) is a term used to label thalassemia patients who do not require lifelong regular blood transfusion for survival, although they may require occasional or even frequent transfusions in certain clinical setting and for definite period of time. It encompasses three clinically distinct forms such as β-thalassemia intermedia, hemoglobin (HB) E/β-thalassemia, and β-thalassemia. Pretransfusion testing includes the following: sample acceptance, checking historical records, patient testing for ABO/D, antibody screening, establishing compatibility, selecting blood components of the correct group and specification, and labeling and issue of blood.
ABO blood grouping includes forward and reverse grouping. Blood group discrepancy occurs when forward and reverse blood grouping do not match with each other.
With the consent of the patient, here we reported the case of a 37-year-old NTDT patient presented with blood group discrepancy with multiple alloantibodies (anti-c and anti-N), who was transfused with three units of O positive c negative, N negative packed red blood cell (PRBC).
| Case Report|| |
A 37-year-old male NTDT patient presented with features of anemia was admitted to the Department of Haematology, SCB Medical College and Hospital, Cuttack, for anemia correction before surgery. The patient had cholelithiasis with symptoms of weakness, breathlessness, and abdominal pain. The patient presented to our department of transfusion medicine for ABO/RhD typing and transfusion support. His laboratory features were HB – 5.9 g/dl, hematocrit – 16%, total white blood cell – 11.6/cumm, Total Platelet Count (TPC) – 121 × 103/cumm, T. Bilirubin – 3.9 mg/dl, D. Bilirubin – 2.6 mg/dl, serum glutamic-oxaloacetic transaminase (SGOT) – 73 IU/Ml, serum glutamic-pyruvic transaminase (SGPT) – 63 IU/Ml, urea – 12 mg/dl, creatinine – 0.8, and alkaline phosphatase – 224. The patient had multiple small calculi in the gall bladder as founded in the ultrasonography. He received the first transfusion at the age of 12 years. Previously, the patient was transfused with two units of cross-matched O-positive PRBC from other blood bank 4 months back and there was no history of transfusion reaction. During that time, his HB was 6.5 g/dl. HB did not rise after transfusion. In fact, he presented with a HB value of 5.6 g/dl.
Two milliliters of EDTA blood and three milliliters of plain vial blood were sent for ABO/RhD typing and cross-matching. Blood grouping including both forward and reverse grouping was done by the standard test tube technique using monoclonal anti-A, anti-B, and anti-D (Tulip Diagnostics, Goa, India) and in-house prepared 5% A, B, and O group pooled red cells respectively. The forward grouping showed O positive, and reverse grouping was found as similar to O grouping with additional reaction with pooled O cell. There was agglutination reaction of the patient's red cell suspension with anti-H lectin from Ulex Europaeus (Tulip Diagnostic, Goa, India) and hence Bombay phenotype was ruled out.
Antibody screening and identification were done using 3-cell panel and 11-cell panel (Ortho Clinical Diagnostics, USA), respectively. We identified the presence of anti-c and anti-N alloantibodies in the patient's serum [Figure 1] and [Figure 2]. The alloantibodies were reacted at immediate spin, 37°C, and antihumanglobulin (AHG) phases in the same strength as shown in [Figure 2]. There was weak reaction(1+) in panel 2 and 11 in AHG phase which may be explained by dosage phenomenon i.e heterozygous expression of antigen N. Phenotype (Ortho Clinical Diagnostics, USA) of the patient's red cell showed the absence of c and N antigen; hence, it indirectly supported the presence of anti-c and anti-N alloantibodies.
|Figure 1: Antigram of antibody screening showing 4+ reaction in panel two and three|
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|Figure 2: Antigram of antibody identification suggestive of anti-c and anti-N|
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Reverse grouping discrepancy was resolved by repeating with c and N antigen negative in-house prepared 5% pooled A, B, and O red cells. The patient was transfused with three units of c and N negative, O Positive AHG cross matched PRBCs and his HB increased to 10.8 g/dl. He was treated with hematinics and antibiotics. Surgery (cholecystectomy) was done as scheduled. We did not find any additional antibodies on follow-up except anti-c and anti-N.
| Discussion|| |
In NTDT, erythropoiesis is ineffective due to the imbalance in the production of α- and β-globin chains. Unstable globin chain tetramers precipitate and undergo oxidation into methemoglobin and hemichromes with eventual separation of heme from globin. The free iron released from heme disintegration in thalassemia erythroid cells eventually catalyzes the formation of reactive oxygen species which leads to oxidation of membrane proteins, structural membrane defects, and exposure of red-cell senescence antigens such as phosphatidylserine causing premature cell death within the bone marrow (ineffective erythropoiesis) or peripheral circulation (hemolysis)., Transfusion therapy is effective in supplying normal erythrocytes and suppressing ineffective erythropoiesis., Our case required transfusion before surgery due to cholelithiasis and showed discrepancy on reverse grouping.
A blood group discrepancy occurs in patients when forward grouping does not match with reverse grouping, usually due to unexpected negative or positive results either in red cell or plasma testing. In our case, there were unexpected positive results with pooled O red cells. These results can be seen with Bombay phenotype, but there was agglutination reaction with anti-H lectin (Ulex europaeus) and hence Bombay phenotype was ruled out. Cold alloantibodies such as anti-M and anti-N reactive with reverse-grouping cells can lead to unexpected positive reactions. Anti-N is usually naturally occurring IgM antibodies which are not active at 37°C. It can generally be ignored in transfusion practice, and if the room temperature incubation is eliminated from compatibility testing and screening for antibodies, antibody will usually not be detected. The anti-N antibody in our cases was IgM type, having a wide thermal amplitude reacting at 37°C and capable of causing hemolytic transfusion reactions (HTRs). ABO discrepancy due to cold alloantibodies (e.g., anti-M) or autoantibodies (e.g., anti-I) reacting with reverse-grouping cells, leading to unexpected positive reactions, has been described in the literature. In our case, we found anti-c and anti-N alloantibodies in antibody identification panel cell and serum testing was resolved by testing reverse grouping panel cell with N-negative pooled cells.
Anti-c, mostly IgG, is clinically the most common Rh antibody after anti-D and is reported to cause hemolytic disease of newborn and delayed HTR as a single or with anti-E antibody. According to the North Indian study, the incidence of RBC alloimmunization in transfused patients is reported to be 3.4% (18/531), with anti-c being the most common (specificity 38.8%). The patient was transfused with three units of c and N antigen negative (O Positive) compatible red blood cell.
| Conclusion|| |
In India, routine pretransfusion testing in many of the peripheral centers does not include antiglobulin phase of cross-matching, which is essential to detect clinically significant IgG antibodies such as Rh, Kell, Kidd, and Duffy. Limited centers have the facility for antibody screening and identification and to provide antigen negative blood to alloimmunized recipients. Prevention strategies for HTR in a known alloimmunized patient include informing the patient about his/her antibody profile and providing him/her a blood bank identity card and most importantly minimizing unnecessary blood transfusion. The blood bank should maintain hospital records of every patient requiring multiple blood transfusions.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Musallam KM, Rivella S, Vichinsky E, Rachmilewitz EA. Non-transfusion-dependent thalassemias. Haematologica 2013;98:833-44.
Weatherall DJ. The definition and epidemiology of non-transfusion-dependent thalassemia. Blood Rev 2012;26 Suppl 1:S3-6.
Musallam KM, Rivella S, Vichinsky E, Rachmilewitz EA. Non-transfusion-dependent thalassemias. Haematologica 2013;98:836.
Musallam KM, Taher AT, Duca L, Cesaretti C, Halawi R, Cappellini MD, et al.
Levels of growth differentiation factor-15 are high and correlate with clinical severity in transfusion-independent patients with β thalassemia intermedia. Blood Cells Mol Dis 2011;47:232-4.
Cazzola M, Finch CA. Evaluation of erythroid marrow function in anemic patients. Haematologica 1987;72:195-200.
Cazzola M, Pootrakul P, Huebers HA, Eng M, Eschbach J, Finch CA, et al.
Erythroid marrow function in anemic patients. Blood 1987;69:296-301.
Daniels G. Other blood groups. In: Roback JD, Grossman BJ, Harris T, Hillyer CD, editors. Technical Manual. 17th
ed. Bethesda, Maryland, USA: American Association of Blood Bank; 2011. p. 416.
Cooling L. ABO, H, and Lewis blood groups and structurally related antigens. In: Roback JD, Grossman BJ, Harris T, Hillyer CD, editors. Technical Manual. 17th
ed. Bethesda, Maryland, USA: American Association of Blood Bank; 2011. p. 272.
Hillman NM. Fatal delayed hemolytic transfusion reaction due to anti-c + E. Transfusion 1979;19:548-51.
Thakral B, Saluja K, Shrma 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.
[Figure 1], [Figure 2]