Hyperhaemolysis syndrome treated with corticosteroids and darbopoietin in a patient with mantle cell lymphoma

摘要

Delayed haemolytic transfusion reaction hyperhaemolysis (DHTR/H) syndrome is a potentially life-threatening condition where there is breakdown of both allogeneic and autologous red cells following a red blood cell transfusion. This causes a drop in haemoglobin to levels below pre-transfusion concentrations associated with a rise in the biochemical markers of haemolysis in the context of a DHTR. This phenomenon was first recognised in patients with sickle cell disease and is often associated with fever and pain (Petz et al, 1997). Only a proportion of patients develop a positive direct antiglobulin test (DAT) and it is rare to detect new alloantibod-ies. This, together with the fact that many patients who develop DHTR/H have a paradoxical reticulocytopenia, suggests that there may be multiple pathogenic mechanisms for DHTR/H including bystander haemolysis (epitope spreading), contact lysis of red cells by activated macrophages and suppression of erythropoiesis (Win et al., 2008).