Sickle Cell Hepatopathy With Acute Hepatic Sequestration and Extreme Hyperbilirubinemia.

Document Type

Article

Publication Date

5-27-2025

Publication Title

Cureus

Abstract

Sickle cell disease (SCD) is a genetic disorder characterized by chronic hemolytic anemia and multi-organ dysfunction. The liver is frequently affected in SCD, with complications spanning from mild hyperbilirubinemia to acute liver failure. Acute hepatic sequestration (AHS), a rare but serious manifestation of sickle cell hepatopathy, can rapidly progress to multi-organ dysfunction syndrome (MODS). Cases with extreme hyperbilirubinemia (bilirubin > 20 mg/dL) remain exceptionally uncommon. A 19-year-old female patient with SCD presented with nausea, vomiting, generalized pain, dysuria, and suprapubic pain. On presentation, her vital signs were stable except for tachycardia (139 beats per minute (bpm)). Physical examination revealed scleral icterus, conjunctival pallor, and right upper quadrant (RUQ) tenderness. Initial laboratory results showed leukocytosis (white blood cell (WBC): 22.7 bil/L), hemoglobin (Hb) of 7.5 g/dL (baseline: 8.5 g/dL), and platelet (PLT) of 178 bil/L. Lactate dehydrogenase (LDH) was at 734 U/L. Liver function tests showed alkaline phosphatase (ALP) at 238 IU/L, aspartate transaminase (AST) at 52 U/L, alanine transaminase (ALT) at 49 U/L, and total bilirubin at 13.4 mg/dL. Abdominal ultrasound showed hepatomegaly (liver span of 19 cm) and gallstones without signs of cholecystitis. Blood cultures (2/2) tested positive for

Volume

17

Issue

5

First Page

e84888

DOI

10.7759/cureus.84888

ISSN

2168-8184

PubMed ID

40575228

Link to Full Text

Share

COinS