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

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.

New Applications of HBOC-201: A 25-Year Review of the Literature

“…Hemoglobin-based oxygen carrier-201 has been allowed by the FDA for use under the expanded access in Jehovah's Witnesses patients who require blood transfusion. HBOC-201 has no cell membrane and lacks cell surface antigens, making HBOC-201 an ideal substitute for blood to save lives when blood is unavailable or blood transfusion is refused for patients with severe autoimmune hemolytic anemia ( 30 , 32 , 33 , 36 , 37 ). Epperla et al demonstrated that early recognition of patient needs and familiarity with the characteristics of HBOC-201 by clinicians are essential for the safe and timely use of HBOC-201 to save the lives of the patients ( 33 ).…”

Hyperhaemolysis Syndrome: A Review of Cases

“…The role of exchange transfusion was believed to be benefi cial as it removes the antibody-coated RBC and circulating bilirubin. Hence, replacing them with RBCs compatible with the patient's serum and providing albumin with new bilirubin binding sites [5,11,19,21]. The role of specifi c immunotherapy has also been described in some cases.…”

Indications for transfusion in the management of sickle cell disease

“…52 In recent years, case reports have described the suc cess ful use of HBOC-201 in the set tings of hyperhemolysis and alloimmunization as well as for Jeho vah's Witnesses, though there were reports of hyper ten sion, elevated liver enzymes, and met he mo glo bi ne mia. [52][53][54] While HBOCs can help to avoid or replace trans fu sion, with out fur ther studies of safety and eff cacy and US Food and Drug Administration approval these agents will have lim ited avail abil ity.…”