Who is eating the red cells?

29 yo F with SCC presenting with a Hct of 13 which did not budge after transfusion and was diagnosed with a rare hematologic complication called Hyperhemolysis Syndrome.  Key points to know about this are:

– the post-transfusion Hct may be unchanged or even lower than pre-transfusion numbers

– mechanisms implicated are: ineffective erythropoiesis, activation of macrophages leading to neocytolysis and bystander hemolysis

– further blood transfusions aggravate the macrophage activation and hemolysis

– treatment: IVIG alongwith steroids and then if absolutely necessary blood transfusion

References: Hyperhemolysis in SCC, CKD in SCD