Acute Hyperhemolysis Syndrome in a Patient with Known Sickle Cell Anemia Refractory to Steroids and IVIG Treated with Tocilizumab and Erythropoietin: A Case Report and Review of Literature

Abstract

Patients with sickle cell anemia often receive multiple red blood cell (RBC) transfusions during their lifetime. Hyperhemolysis is a life-threatening phenomenon of accelerated hemolysis and worsening anemia that occurs when both transfused RBCs and autologous RBCs are destroyed. The level of hemoglobin post-transfusion is lower than pre-transfusion levels, and patients are usually hemodynamically unstable. Hyperhemolysis must be differentiated from a delayed hemolytic transfusion reaction during which destruction of transfused RBC is the cause of anemia. Hyperhemolysis syndrome can be differentiated into acute (within seven days) and chronic forms (after seven days) post-transfusion. The authors present a case of acute hyperhemolysis syndrome in a patient with sickle cell anemia refractory to steroids and IVIG, which are the treatment of choice. The patient was treated with tocilizumab, combined with supportive measures of erythropoietin, iron, vitamin B12, and folate.

Successful treatment with plasma exchange in life‐threatening hyperhemolytic syndrome unrelated to sickle cell disease

“…Targeting macrophage activation using Tocilizumab has recently emerged as a treatment option in multiple case reports published after the occurrence of our two cases. 18 Splenectomy has been utilized in some cases of HHS, 24 although this has not always resulted in resolution of hemolysis. 25 In our patients, the low levels of hemoglobin and inability to transfuse the patients prohibited any attempt at surgery in the initial stages of the disease.…”

“…16 Beyond supportive care and avoiding further transfusions, treatment for severe cases may include immunosuppression using steroids, intravenous immunoglobulin (IVIG) and rituximab, as well as the anti-CD5 antibody, eculizimab, 17 and anti-IL-6 treatment (Figure 1). 18 We report here two cases of severe life-threatening HHS refractory to immunosuppression who responded to plasma exchange followed by splenectomy.…”

“…Beyond supportive care and avoiding further transfusions, treatment for severe cases may include immunosuppression using steroids, intravenous immunoglobulin (IVIG) and rituximab, as well as the anti‐CD5 antibody, eculizimab, 17 and anti‐IL‐6 treatment (Figure 1). 18 …”

Transfusion therapy for sickle cell disease: what’s new?

“…The 2020 ASH SCD transfusion support guidelines suggested immunosuppressive therapy over no immunosuppressive therapy for DHTRs, including the use of IVIg, steroids, rituximab, and/or eculizumab. Since that time, case reports have described individuals with SCD and HTRs successfully treated with an interleukin-6 (IL-6) receptor antagonist (tocilizumab [Actemra]) [7][8][9][10] . The binding of tocilizumab to the IL-6 receptor inhibits, among other things, IL-6 mediated macrophage activation.…”

Hyperhaemolysis Syndrome: A Review of Cases

“…Hyperhaemolysis syndrome is a rare life-threatening delayed complication of blood transfusion [1]. This hyperhaemolytic reaction is characterized by the destruction of both transfused and autologous erythrocytes.…”