Autoimmunization in Thalassemia: A case report with review of literature

  • Sangeeta Pahuja Regional Blood Transfusion Center, Department of Pathology, Lady Hardinge Medical College& Kalawati Saran Children’s Hospital, New Delhi , India-110001
  • Geetika Sharma Regional Blood Transfusion Center, Department of Pathology, Lady Hardinge Medical College& Kalawati Saran Children’s Hospital, New Delhi , India-110001
  • P. Lalita Jyotsna Regional Blood Transfusion Center, Department of Pathology, Lady Hardinge Medical College& Kalawati Saran Children’s Hospital, New Delhi , India-110001
  • Richa Chauhan Regional Blood Transfusion Center, Department of Pathology, Lady Hardinge Medical College& Kalawati Saran Children’s Hospital, New Delhi , India-110001
Keywords: Thalassemia major, Red Cell Autoimmunization, Autoantibodies.

Abstract

Alloimmunization has been variably reported worldwide in multi transfused thalassemic patients and leads to delay in issue of compatible blood. However, there is paucity of literature on the frequency of red cell auto-immunization in thalassemics, particularly of Indian origin.We present a case of 4 year old female child, known case of thalassemia major, who presented to the pediatric emergency with impending congestive heart failure. The EDTA sample showed auto-agglutination on naked eye at room temperature which persisted on incubating the sample at 37°C.There was discrepancy in forward and reverse blood grouping at room temperature which was resolved by extended blood grouping at 3 temperatures (4°C, 22°C & 37°C) and patient’s blood group was confirmed to be B positive. The polyspecific direct antiglobulin test (DAT) was strongly positive with 4+ reaction and DAT profiling revealed presence of IgG and C3d on red cells.The antibody screening and identification [Biorad ID Diacell I, II & III and Diapanel (11 cell panel)] respectively showed a panagglutinating reaction in Coomb’s phase at 37°C. The titers for IgM antibody in saline phase were found to be  clinically non significant.Multiple units put up for the cross match were all incompatible. However, she was given profile matched (“c”neg, “E”neg and “Kell”neg) least incompatible blood slowly, under strict clinical supervision after the written consent from the clinician.Thus, guidelines for transfusion policy for thalassemic child need to be formulated to minimize the risk of immunization. Rh and Kell matched blood for thalassemic children (better match) has been shown to reduce the rate of immunization in thalassemic children.

References

1. Weatherall D. The Thalassemias: The Role of Molecular Genetics in an Evolving Global Health Problem. American Journal of Human Genetics 2004;74(3):385-392.
2. Singer ST, Wu V, Mignacca R, Kuypers FA, Morel P, Vichinsky EP. Alloimmunization and erythrocyte autoimmunization in transfusion-dependent thalassemia patients of predominantly asian descent. Blood. 2000 Nov 15;96(10):3369-73.
3. Dhawan HK, Kumawat V, Marwaha N, Sharma RR, Sachdev S, Bansal D, Marwaha RK, Arora S. Alloimmunization and autoimmunization in transfusion dependent thalassemia major patients: Study on 319 patients. Asian J Transfus Sci. 2014 Jul;8(2):84-8.
4. Pahuja S, Pujani M, Gupta SK, Chandra J, Jain M. Alloimmunization and red cell autoimmunization in multitransfused thalassemics of Indian origin. Hematology. 2010 Jun;15(3):174-7.
5. Noor Haslina MN, Ariffin N, Illuni Hayati I, Rosline H. Red cell immunization in multiply transfused Malay thalassemic patients. Southeast Asian J Trop Med Public Health. 2006 Sep;37(5):1015-20.
6. Hassan K, Younus M, Ikram N, Naseem L, Zaheer HA.Red Cell Alloimmunization in Repeatedly Transfused Thalassemia Major Patients. International Journal of Pathology. 2004; 2(1):16-19.
7. Ameen R, Al-Shemmari S, Al-Humood S, Chowdhury RI, Al-Eyaadi O, Al-Bashir A. RBC alloimmunization and autoimmunization among transfusion-dependent Arab thalassemia patients. Transfusion. 2003 Nov;43(11):1604-10.
8. Michail-Merianou V, Pamphili-Panousopoulou L, Piperi-Lowes L, Pelegrinis E, Karaklis A. Alloimmunization to red cell antigens in thalassemia: comparative study of usual versus better-match transfusion programmes. Vox Sang. 1987;52(1-2):95-8.
9. Chaudhary RK, Das SS. Autoimmune hemolytic anemia: From lab to bedside. Asian J Transfus Sci. 2014 Jan;8(1):5-12.
Published
2016-10-03
Issue
Vol 3 No 4 (2016)
Section
Case Report

Authors who publish with this journal agree to the following terms:

  1. Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
  2. Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
  3. Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access at http://opcit.eprints.org/oacitation-biblio.html).