Hyperhemolytic Crisis Following Transfusion in Sickle Cell Disease With Acute Hepatic Crisis: A Case Report

Abstract

Hyperhemolysis syndrome (HHS) is a catastrophic unpredictable consequence of blood transfusion in sickle cell disease. It leads to further drop in hemoglobin via immune mechanisms complicating a hospital course and prolonging length of stay. Although sickle cell patients receive multiple transfusions throughout their disease course, this condition remains underreported by health care professionals or misinterpreted for other sickle cell crises. We present a similar case highlighting the importance of early recognition of HHS and judicious blood transfusion in sickle cell disease patients to avoid such a complication.

A Case Report of Hyperhemolytic Syndrome in Sickle Cell Disease, with a Special Focus on Avoiding the Use of Transfusions

“…EPO treatment was also applied after a cost/benefit assessment. In addition to the above, the literature data demonstrate that rituximab, a monoclonal antibody against CD20, is a good choice to treat the underlying autoimmune condition and could also rapidly re-establish the reticulocyte count [17]. Menakuru et al administered tocilizumab, a monoclonal antibody against IL-6 (interleukin-6) and a targeted therapy used in autoimmune conditions, to treat patients with severe acute HHS, supporting the macrophage activation mechanism [22].…”

“…The exact mechanism of HHS is not established but there are different theories to explain severe hemolysis. One theory is the "bystander complement activation"; after transfusion, both the donor and recipient RBCs are destroyed by a non-specific antibody activating the complement system [17,19], which leads to a lower post-transfusion Hgb level. This mechanism could be the background of our case.…”

“…This mechanism could be the background of our case. The second mechanism is "suppressed erythropoiesis", which explains acute reticulocytopenia [17,19], just like the immune-mediated hemolysis of the RBC precursors. Moreover, sickled RBC highly expresses phosphatidylserine, drawing the activated macrophages' attention to these cells to be removed from circulation [17].…”

“…Treatment of HHS is avoiding the use of transfusions; if transfusions are still necessary, then premedication with corticosteroids or intravenous immunoglobulin (IVIG) could be used. Based on the previous findings, in some case reports, HHS was treated with IVIG (0.5 g/kg/day for 5 days) and methylprednisolone 0.5 g/day for 2 days [16,17].…”