What Do We Mean By 'Hyperhaemolysis' And What Is The Cause?

摘要

The prefix "hyper" means over, above, beyond, or excessive or above normal, so "hyperhaemolysis" was first used in haematology to describe severe haemolytic anaemia (usually intravascular lysis) associated with immune and non-immune haemolytic anaemias. From the early 1990s, the term started to be used to describe a certain type of life-threatening haemolytic transfusion reaction (HTR), associated with sickle cell disease (SCD). The main distinctive characteristic was that the haemoglobin level fell below the pretransfusion level, suggesting that both transfused and the patient's own red blood cells (RBCs) were being destroyed. There are now a large number of publications describing this event, suggesting what might be causing it, and how to treat it. More recently, the use of terms I now prefer more than the single word "hyperhaemolysis" are: "hyperhaemolytic transfusion reaction" (Sirchia etal, 1997; Win etal, 2010); or "hyperhaemolysis syndrome" (Darabi & Dzik, 2005). I think that the former is preferable as it associates the syndrome with a HTR. Ballas & Marcolina (2006) make the point that "hyperhaemolysis"can also occur in some patients with SCD due to infection with malarial parasites, babesia and mycoplasma; G6PD deficiency; and autoimmune haemolytic anaemia. Two recent publications (Taylor etal., 2008; Hebbel, 2010) discuss hyperhaemolysis in a much broader sense than used by blood transfusion medicine scientists. I strongly recommend the "tour de force" review by Hebbel to gain a much wider viewpoint on hyperhaemolysis.