Manual RBC exchange transfusion in a patient of sickle-beta thalassemia syndrome presenting in crisis: a case report

Authors

  • Girija Nandini Kanungo Department of Transfusion Medicine, IMS and SUM Hospital, Bhubaneswar, Odisha, India
  • Bibudhendu Pati Department of Transfusion Medicine, IMS and SUM Hospital, Bhubaneswar, Odisha, India
  • Hemlata Das Department of Transfusion Medicine, IMS and SUM Hospital, Bhubaneswar, Odisha, India
  • Suman Sudha Routray Department of Transfusion Medicine, IMS and SUM Hospital, Bhubaneswar, Odisha, India

DOI:

https://doi.org/10.18203/2349-3933.ijam20172286

Keywords:

Exchange transfusion, Manual red cell exchange, Sickle cell anaemia

Abstract

Sickle cell disease and beta thalassemia are caused by abnormal haemoglobin (Hb) derived from mutation of the HBB gene encoding beta-globin. Compound heterozygous status for both mutations results in HbS/beta thalassemia (Sickle- beta thalassemia). Vaso-occlusive phenomena and haemolysis are the clinical hallmarks and major causes of mortality. Here we report a case of successful reduction of HbS level by manual RBC exchange transfusion. Capillary zone electrophoresis showed the case to be Sickle-beta thalassemia. A total of   3 units of 450ml whole blood units were used for manual exchange transfusion done in 2 sittings on consecutive days. Preexchange HbS level was 80.9% of total Hb. HbS level after 24 hours of the second procedure was 44%. In the absence of facility to conduct automated RBC exchange by a cell separator, to reduce HbS in patients presenting with acute complications of SCD and in patients with Vaso-occlusive Crisis, previous stroke, manual RBC exchange can provide a better relief.

References

Choi R, Song J, Jang HK, Kim S, Jung CW, Park HD, et al. A case of red blood cell exchange transfusion in a patient with haemoglobin s/beta thalassemia; Korean J Blood Trans. 2012;23(3):256-61.

Jaeckel R, Thieme M, Czeslick E, Sablotzki A. The use of partial exchange blood transfusion and anaesthesia in the management of sickle cell disease in a perioperative setting: two case reports. J Med Case Rep. 2010;4(1):82.

de Montalembert M, Aout J. Exchange Transfusion in sickle cell disease. Haematologie. 2007;13(4):243-9.

Escobar C, Mascarenhas MI, Moniz M, Nunes P, Silvestre C, Abbadesso C, et al. Red cell partial exchange transfusion in sickle cell disease: A paediatric Intensive Care Unit Experience; Paediatr Crit Care Med. 2014;15(4):150.

Hoffman R, Benz E, Silberstein L, Heslop H, Weitz J, Anastasi J. Sickle cell diseases: Clinical Features and Management. In: Hematology: Basic principles and practice. 6th ed. Philadelphia; 2012:555.

Kuo KHM, Ward R, Kaya B, Howard J, Telpher P. A comparison of chronic manual and automated red blood cell exchange transfusion in sickle cell disease patients. Br J Hematol. 2015;170(3):425-28.

Downloads

Published

2017-05-23

Issue

Section

Case Reports