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.

Keywords: alloimmunization; hepatic crisis; hyperhemolytic crisis; sickle cell disease; transfusion related complications.

Figure 1. CTA chest axial bone window showing bilateral erosions and collapse of humeral heads consistent with avascular necrosis.

CTA: CT angiography

Figure 2. CTA chest axial lung window showing right lower pleural effusion with possible atelectasis and consolidation.

CTA: CT angiography

Figure 3. US abdomen showing hepatomegaly measuring 22.8 cm in longitudinal dimension.

Figure 4. Destruction of native and transfused red cells by activated macrophages due to exposure to phosphatidylserine.

HbSS: sickle cell hemoglobin; α4β1: hemoglobin subunits in a sickle cell; VCAM-1: vascular cell adhesion molecule-1; ICAM-4: intercellular adhesion molecule-4; CD: cluster of differentiation The figure is obtained with permission from Madu et al. (2021) [7].