|Year : 2019 | Volume
| Issue : 1 | Page : 111-113
How a transfusion medicine consultation became a life-saving intervention for an E-Beta thalassemia patient: A case report from Kolkata, West Bengal
Biplabendu Talukdar1, Soumyarupa Majee1, Prasun Bhattacharya1, Subrata Das2, Rony Roy Chaudhury2, SK Sabib2
1 Department of IHBT, Medical College, Kolkata, West Bengal, India
2 Peoples Blood Bank, Kolkata, West Bengal, India
|Date of Web Publication||22-Apr-2019|
Dr. Prasun Bhattacharya
Department of IHBT, Medical College, Kolkata, West Bengal
Source of Support: None, Conflict of Interest: None
Bedside transfusion practice is a challenge for Transfusion Medicine practitioners in multi-transfused patients who have irregular antibody(s) against donors red cell antigens. Apart from ABO and Rh, there are more than 300 identified blood group antigens present on the surface of red cell membrane. We present a patient of E-Beta thalassemia in 36 weeks of gestation who is nontransfusion-dependent thalassemic. She had received two units of concentrated red blood cells (RBCs) 2 years back during the birth of her first child by cesarean section. She was severely pale when she attended the obstetrics outpatients' department for her second pregnancy. Urgent transfusion of two units of ABO-matched packed RBCs worsened the anemia and jaundice. Instead of hemoglobin increment, fall of Hb with cola colored urine occurred within a short period. Antibody identification was done, and antibody negative red cell transfusion was given which helped the obstetrician for safe delivery and well-being of the mother.
Keywords: Cesarean section, direct antiglobulin test, indirect antiglobulin test, nontransfusion dependent thalassemia, red blood cell
|How to cite this article:|
Talukdar B, Majee S, Bhattacharya P, Das S, Chaudhury RR, Sabib S K. How a transfusion medicine consultation became a life-saving intervention for an E-Beta thalassemia patient: A case report from Kolkata, West Bengal. Glob J Transfus Med 2019;4:111-3
|How to cite this URL:|
Talukdar B, Majee S, Bhattacharya P, Das S, Chaudhury RR, Sabib S K. How a transfusion medicine consultation became a life-saving intervention for an E-Beta thalassemia patient: A case report from Kolkata, West Bengal. Glob J Transfus Med [serial online] 2019 [cited 2020 Aug 13];4:111-3. Available from: http://www.gjtmonline.com/text.asp?2019/4/1/111/256738
| Introduction|| |
Indirect antiglobulin test (IAT) detects the presence of irregular antibody(s) against human red blood cell (RBC) antigens. A positive IAT determines whether there are irregular alloantibodies in the mother's blood, commonly against fetal red cell antigens. Rh alloimmunization occurs when maternal immune system is sensitized to Rh erythrocyte surface antigens.
Alloimmunization may also occur against donor red cells when a nonself antigen is introduced in an immunocompetent recipient to evoke an immune response. The development of immune response depends on several factors. The immune response against carbohydrate antigens is usually thymus independent. Carbohydrate antigens directly stimulate B cells to synthesize antibodies without the involvement of helper T cells resulting in the majority of cases in the production of immunoglobulin M antibodies.,, The most important irregular RBC alloantibodies in daily transfusion practice, in terms of frequency of occurrence, are directed towards the Rh (anti-D, C, E, c and e), Kell (anti-K), Duffy (anti-Fya and Fyb), Kidd (anti-Jka and Jkb), and the MNS (anti-M, S and s) blood group systems.
The most common causes of maternal Rh alloimmunization are blood transfusion and antepartum or intrapartum fetomaternal hemorrhage (abdominal trauma, abortion, ectopic pregnancy, invasive obstetric procedures, placental abruption, and external cephalic version). The evaluation of maternal ABO blood group, Rh type, and anti-D antibodies (indirect Coombs test) should be performed at the prenatal visits.
| Case Report|| |
Here, we present a case of a 20-year-old mother in her 36 weeks of gestation with a previous cesarean section (CS) and a known case of E–Beta-thalassemia. She came in her second pregnancy to the obstetrics department at an outside hospital. She had received two units of red cell transfusion during the previous CS (2 years back) and had delivered a healthy baby. During the physical examination, she appeared grossly pale, mildly icteric and had moderate splenomegaly. Her laboratory examination revealed: blood Group: B Rh (D) positive, hemoglobin (Hb) 6.9 g/dl, reticulocyte 1.5%, total bilirubin 2 mg/dl, indirect bilirubin 1.2 mg/dl, and lactate dehydrogenase (LDH) 2750 U/L. Her direct antiglobulin test (DAT) and IAT were negative as reported from an outside laboratory.
After admission in the said hospital, she was transfused two units of B Rh (D) positive red cells. However, posttransfusion Hb fell to 4.7 g/dl with further increase in jaundice as well as LDH level. She was then referred to the Department of Transfusion Medicine, Medical College Hospital for transfusion refractoriness and deterioration of her general condition posttransfusion. Her Hb level was 3.2 g/dl when she was referred to us.
Examination of her EDTA and clotted blood samples showed grossly icteric plasma/serum on visual inspection. Blood group was B Rh D positive, DAT  was strongly positive, IAT was strongly positive with pooled O cells. Her auto control was negative. On further DAT screening, strong positivity of immunoglobulin G specificity was seen with a titer >300 (BioRad, Switzerland). She was positive on antibody screening tests in cell panels I and III (Dia cell Biorad, Switzerland). This pattern suggested that antibody had probably developed against C, Fy a, Jk b antigens. Due to the unavailability of commercial antibody identification panel cells, we used in-house freshly prepared antibody identification panels. The possible antibody was anti “C.” The concerned obstetrician was immediately informed about the test results and its limitations. The transfusion of “C” negative red cells were undertaken under the supervision of transfusion medicine team along with the treating facility. A high-risk consent was obtained before transfusion. The transfusion was monitored, and it was uneventful. There was a posttransfusion hemoglobin increment to 6.4 gm/dl after 24 h. Later on, she was transfused with another 3 units of compatible packed RBCs (PRBCs) with R2 phenotype, and a posttransfusion Hb increment to 9.5 g/dl was noted. She successfully delivered a healthy baby by CS at 38 weeks of gestation.
| Discussion|| |
RBC allosensitization remains a significant clinical challenge in patients with hemoglobinopathies who would otherwise benefit from transfusion. The major cause of alloimmunization in patients with sickle cell disease and thalassemia is exposure to foreign RBC antigens. Delayed hemolytic transfusion reaction which results from the interaction between recipient alloantibodies and target antigens on donor RBCs, present a potentially life-threatening complication of transfusion therapy in sickle cell disease but are probably under-recognized.
A case was reported at Noble Hospital, Maharashtra, India, where a patient was alloimmunized due to past blood transfusions for sickle cell anemia. They experienced severe hemolysis in the patient due to difficulty in cross-matching all red cell antigens in donor and antibodies against the K, E, c. jk b antigens in the patient.
In recent years, the Food and Drug Administration in the USA has reported irregular RBC alloantibodies as a major cause of fatal hemolytic transfusion reactions, and they are considered the second major cause of transfusion-related deaths. Alloimmunization may lead to clinically significant transfusion reactions and decreased survival of RBCs. A study from India showed that the most common alloantibody identified was anti-E (11/44 cases) followed by anti-C (6/44 cases). Of 842 patients, alloantibodies were detected in 5.22% of patients.
The presence of C antigen in our population is 92%, so approximately 60 units of PRBCs were required to be screened to find the five “C” negative units for this patient. The negative past records of DAT and IAT may have been due to the variation of sensitivities in the methodology or any technical error.
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.
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