S2741 Sickling Manifestations: A Challenging Case of Sickle Cell Hepatopathy

Abstract

Introduction: Sickle cell hepatopathy (SCH) is an entity describing a pattern of liver injury seen in an estimated ∼10% of patients with sickle cell disease (SCD). It is more prevalent in the Sub-Saharan African region and South-East Asia. This entity is classified according to its clinical presentation into acute and chronic which differs in clinical diagnosis and management. Case Description/Methods: A 51-year-old African American male with SCD presented to our ER with complaint of joint pain and shortness of breath. Patient-reported progressive bilateral lower extremity arthralgia, sharp, 8/10 in severity for 1 week. Reported Dyspnea on exertion. Recurrent sickle cell crises were noted. The patient recently discontinued hydroxyurea because of non-healing cutaneous ulcerations. Vitals marked hypoxia at 87%, otherwise stable. Physical examination revealed icteric sclerae with bilateral lung crackles prominent at the bases. Labs showed hemoglobin at 3.0 g/dl, HB S 28.7% with reticulocyte count at 17.41%. Chemistry panel showed alkaline phosphatase (ALP) at 209 U/L, Total bilirubin at 7.0 mg/dl with direct bilirubin at 4.7 mg/dl. Gamma-glutamyl transpeptidase (GGT) was normal. Chest Xray showed bilateral multifocal infiltrates. Abdominal ultrasound was unremarkable. Computed tomography of the chest showed interspersed ground-glass opacities. Hepatology was consulted for assessment of elevated ALP and Total bilirubin. Clinical suspicion was (SCH). Patient was given multiple blood transfusions. The need for liver transplant (LT) was investigated and deemed not necessary. Pathology was reviewed. Discussion: (SCH) is included in the differential of liver injury, therefore, identifying this entity from other causes of liver disease is important. It is more prevalent in the US now. Hence, an urgent need to find new treatment options in addition to emergent blood transfusion and LT is required. Iron quantification and determining need for chelation therapy is crucial and affects long-term outcomes. LT in SCD patients is controversial due to high risk of post-transplant complications. More studies and international registries are needed to expand our knowledge of the benefits and risks of LT in patients with SCD. We anticipate that (SCH) will become a burden on the health care system in the aging population of SCD patients as they will require more blood transfusions. With a current national shortage supply, we fear future complications will include an increase in mortality rates within this patient population.Figure 1.: a (H&E stain 400x) showing sickled RBC in the sinusoids. b (H&E stain 40x) The lobules show marked sinusoidal dilatation and congestion with elongated RBCs, more pronounced in zone 3 with extravasated RBCs. c (Trichrome stain 200x) Trichrome stain shows diffuse sinusoidal fibrosis, portal, periportal with bridging.