Use of the blood substitute HBOC‐201 in critically ill patients during sickle crisis: a three‐case series

Abstract

BACKGROUND Red blood cell (RBC) transfusion is an important treatment modality during severe sickle cell crisis (SCC). SCC patients who refuse, or cannot accept, RBCs present a unique challenge. Acellular hemoglobin (Hb)‐based oxygen carriers (HBOCs) might be an alternative for critically ill patients in SCC with multiorgan failure due to life‐threatening anemia. HBOC‐201 (HbO2 Therapeutics) has been administered to more than 800 anemic patients in 22 clinical trials, but use of any HBOCs in critically ill sickle cell patients with organ failure is exceedingly rare. In the United States, HBOC‐201 is currently only available for expanded access. CASE REPORT We report three cases of HBOC‐201 administered to critically ill sickle cell disease patients in SCC with multiorgan failure, either who refused RBCs (Jehovah's Witnesses) or for whom compatible RBCs were not available. RESULTS Two patients received more than 20 units of HBOC‐201, while the other received 6. The 27 units used in the third case equals the largest volume a patient has successfully received to date. All three patients survived to hospital discharge. CONCLUSION These reports suggest that blood substitutes such as HBOC‐201 can provide an oxygen bridge in SCC with multiorgan failure, until corpuscular Hb levels recover to meet metabolic demand, and highlight the compelling biochemical properties that warrant further investigation.

Ex situ normothermic machine perfusion of donor livers using a haemoglobin-based oxygen carrier: a viable alternative to red blood cells

“…Potential side effects, such as described after in vivo clinical transfusion, are less likely to occur when an HBOC-based perfusion solution is used for ex situ NMP of donor livers, because the donor liver is extensively flushed prior to implantation. Moreover, although HBOC may not be as effective as RBC in treating severe anaemia in clinical trials, postmarketing experience in South Africa has recently resulted in FDA-approved clinical application for life-threatening anaemia in patients unable or unwilling to undergo RBC transfusion [3,4].Several groups have reported experience with an HBOC-201 (HbO 2 therapeutics)-based perfusion fluid for ex situ liver machine perfusion, all of which showed favourable results [5][6][7]. Fontes et al were the first to report on the use of an HBOC-based perfusion fluid in machine perfusion [5].…”

“…Potential side effects, such as described after in vivo clinical transfusion, are less likely to occur when an HBOC-based perfusion solution is used for ex situ NMP of donor livers, because the donor liver is extensively flushed prior to implantation. Moreover, although HBOC may not be as effective as RBC in treating severe anaemia in clinical trials, postmarketing experience in South Africa has recently resulted in FDA-approved clinical application for life-threatening anaemia in patients unable or unwilling to undergo RBC transfusion [3,4].…”

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

“…The use of Hemopure has been reported in a limited number of patients with sickle cell anaemia during sickle cell crises and also in a small number of asymptomatic volunteers with sickle cell anaemia. In both subsets, favourable physiologic benefits of Hemopure were described and there were no adverse consequences …”

“…In both subsets, favourable physiologic benefits of Hemopure were described and there were no adverse consequences. 11,12 We also describe the use of bortezomib as a therapeutic agent in the treatment of HHS. Bortezomib was utilized to down-regulate the immune system which is felt to be a major driver in the pathogenesis of HHS.…”

Reply to “Ex situ normothermic machine perfusion of donor livers using a haemoglobin-based oxygen carrier: a viable alternative to red blood cells”

“…This is however different from the use of HBOC in vivo, which is shown to increase the risk of death and myocardial infarction [3]. The mentioned clinical application in USA was only granted through an expanded access programme and additional institutional review board approval was required for such a use [4]. Of note, HBOC (Hemopure) is available under investigational status in USA, and is not FDA approved [5].…”