Successful management of the potentially fatal hyperhaemolysis syndrome of sickle cell anaemia with a regimen including bortezomib and Hemopure.

WHAT IS KNOWN AND OBJECTIVE

Hyperhaemolysis syndrome (HHS) of sickle cell anaemia (SCA) is a life-threatening condition characterized by accelerated destruction of red blood cells typically following blood transfusions. Optimal treatment strategies have not been determined; therefore, reports utilizing novel therapies are needed.

CASE DESCRIPTION

A 19-year-old African American man with SCA experienced HHS following a partial red cell exchange transfusion. He was treated with methylprednisolone, rituximab, darbepoetin, Hemopure and bortezomib, with resolution of the syndrome.

WHAT IS NEW AND CONCLUSION

The HHS of SCA is thought to be immune-mediated even in the absence of detectable red cell alloantibodies. New therapies, including bortezomib and Hemopure, may be useful in this syndrome.