Hyperhaemolysis in Sickle Cell Disease Is Not Necessarily a Transfusion Reaction

Hyperhaemolysis is a rare life threatening complication in sickle cell disease with rapidly dropping haemoglobin, intravascular haemolysis and haemoglobinuria leading to multi organ failure and death. The literature reports that hyperhaemolysis in sickle cell disease is a complication of red cell transfusion (Aragona et al., 2014 J. Pediatr. Hematol. Oncol.) and suggests management based on with holding further transfusion to avoid aggravating the haemolysis and using immunosuppression (Win 2009 Expert Rev. Hematol.). In the literature, all cases of hyperhaemolysis in addition to a recent blood transfusion, were in or had had a recent sickle cell crisis.

We report a case of life threatening Hyperhaemolysis in a 5 year old child following a sickle cell crisis who had never previously been transfused. We suggest that, at least in this case, the hyperhaemolysis cannot be transfusion related. The theoretical case for management of withholding transfusion may not be sound and potentially dangerous.

A female child with known sickle cell disease presented with temperature and chest pains, she had a Hb 72g/L (stable over a few years). She initially improved with oxygen, fluids and antibiotics. 36 hours after admission she acutely deteriorated with increasing pallor and dropping oxygen saturations. She started passing frank red urine which initially was considered to be haematuria but on investigation was haemoglobinuria. Her Hb dropped to 47g/L with no evidence of blood loss. Within hours of developing haemoglobinuria she required intensive care for respiratory support. She rapidly developed multi-organ failure requiring oscillatory ventilation, inotropes, and haemofiltration for renal support.

She was managed with emergency red cell transfusion (her first ever) and within 12 hours of haemoglobinuria received a full red cell exchange transfusion. There were ongoing antibiotics for clinical respiratory infection and she was later confirmed to have influenza B. No steroids or other immune suppression were given. There was no evidence of acute bleeding to explain a drop in haemoglobin at any point. With maximum intensive care support including further transfusions she gradually improved and has made a full recovery. No deterioration was observed following transfusion. She has remained well since. She is now 13 years old and following such a dramatic episode she has remained on a transfusion programme with successful oral iron chelation. She has not experienced any further episodes of hyperhaemolysis and no red cell antibody has been detected at any time.

This case demonstrates that hyperhaemolysis in sickle cell disease does not require a previous transfusion. We suggest that it is possible the previous reported cases are also not due to blood transfusion but are an acute form of haemolysis seen on the background of a chronic haemolytic disease. An increase in the rate of haemolysis may be related to other acute complications of sickle cell disease.

We propose that the optimum management of hyperhaemolysis should include full supportive care including maintaining haemoglobin by transfusion. Immunosuppression in this case could have led to a worse outcome as influenza pneumonia was the likely initial trigger of the episode.