Chronic blood transfusion in a long-survivor sickle cell anaemia patient

Abstract

Background: Multiple blood transfusions, with consequent iron overload, are inevitable in Sickle Cell Anaemia (SCA) patients who suffer from chronic haemolytic process that is frequently complicated by hyper-haemolytic crisis.

Objective: This paper reports the long term effects of blood transfusion in a long surviving SCA patient and to emphasize the need for exchange blood transfusions, regular iron studies and the need for chelation therapy in order to avoid organ failures.

Materials & Method: The hospital record of the patient was reviewed.

Results: This case typifies one of the extremes of transfusion requirement in a sickle cell anaemia patient. She survived multiple crises and complications of the disease up to the age of sixty one years, but in the course of the disease, she developed chronic kidney disease that warranted multiple episodic and chronic blood transfusions. The associated hypertension and subsequent heart failure led to her sudden death. Altogether, she received 40 units of blood, amounting to about 8,800 mg of exogenous iron.  Iron overload was therefore a possible contributory factor to organ failures and death. However, patient's family refused post mortem examination which could have been helpful to determine iron load in the liver, heart and the bone marrow retrospectively.

Conclusion: In view of the complications of multiple blood transfusions with consequent iron overload in SCA patients, the use of artificial blood, regular use of hydroxyurea, iron chelation therapy when indicated will go a long way in improving survival in SCA patients.