Impact of Glucose-6-phosphate Dehydrogenase Deficiency on Sickle Cell Anaemia Expression: A Case Report

Abstract

Sickle cell anaemia and Glucose-6-phosphate dehydrogenase deficiency have anaemia as major clinical consequence. The two disorders are rarely co-expressed in a patient. However, the pathological co-existence of both disorders tends to worsen and aggravate the clinical presentation in affected individuals. We report a diagnosis of the co-inheritance of these two disorders in a 43 year old man who was diagnosed with SCA at childhood. He was managed in a secondary health care facility since child hood. He has had multiple blood transfusions on account of repeated episodes of haemolytic anaemia which was solely attributed to SCA. He was referenced our center on account of recurrent severe heamolytic anaemia with a PCV of 9%. In the preceding 2 months to detecting his G6PD deficient status, he was having monthly blood transfusions on account of severe anaemia. Following the detection of his G6PD deficiency status and appropriate intervention with glutathione and selenium supplements and counseling to avoid exposure to oxidizing agents, he had a respite in the frequency of acute episodes of haemolysis necessitating blood transfusion, as well as an improvement in his steady state PCV of 16%. This case report underscores the importance of routine screening for G6PD status in patients with SCA so as to institute appropriate measures to reduce the worsening of incidence of acute episodes of haemolysis and the need for recurrent blood transfusions on account of this.