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Case Reports
. 2019 Oct;44(5):815-818.
doi: 10.1111/jcpt.12998. Epub 2019 Jun 25.

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

Stacy S Epstein  1 , Terence J Hadley  1
Affiliations
Case Reports

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

Stacy S Epstein et al. J Clin Pharm Ther. 2019 Oct.

Abstract

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.

Keywords: Hemopure; bortezomib; hyperhaemolysis syndrome; sickle cell anaemia.

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References

REFERENCES

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