SHM Converge Scientific Abstract ePoster Gallery-Livin' on a Prayer- A Case of Sinusoidal Obstruction Syndrome

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A 26-year-old man with X-linked agammaglobulinemia on monthly IVIG presented with abdominal pain and jaundice. Workup showed portal hypertension on CT and MRI without significant liver surface nodularity or clear cirrhosis. Laboratory testing revealed cholestatic and synthetic liver dysfunction with transaminitis, elevated alkaline phosphatase, hyperbilirubinemia (total bilirubin 6.5–7.5), INR 1.75, and pancytopenia.<br /><br />An extensive evaluation for other causes of liver disease was negative, including autoimmune markers (ANA, anti-smooth muscle, antimitochondrial, anti-soluble liver enzyme antibodies), viral hepatitis A/B/C, HIV, EBV, CMV, HSV, AFB, Wilson disease, alpha-1 antitrypsin deficiency, and alcohol use markers.<br /><br />The imaging and clinical picture suggested noncirrhotic portal hypertension, and pathology demonstrated findings consistent with sinusoidal obstruction syndrome: hepatocyte hydropic change with foamy cytoplasm, sinusoidal congestion with red blood cells, sinusoidal dilatation, and atrophic hepatocyte plates. Sinusoidal obstruction syndrome is typically associated with chemotoxic injury, but this case raises concern for a possible association with X-linked agammaglobulinemia.<br /><br />This case highlights that X-linked agammaglobulinemia may be associated with unusual liver injury patterns and noncirrhotic portal hypertension. Further study is needed to define the hepatic manifestations of this immunodeficiency and to identify potential risk factors and treatments, especially because sinusoidal obstruction syndrome can progress rapidly and may be fatal.

Asset Subtitle

Manile A. Dastagir

Meta Tag

Author List Manile A. Dastagir, Michelle Loftus, Shalin Shah

Category Clinical Vignettes

Concept Sinusoidal Obstruction

Concept Non-cirrhotic Portal Hypertension

Concept Abdominal Pain

Concept Jaundice

Concept Hyperbilirubinemia

Distinguished Non-Finalist

Presenter Organization Northwell North Shore University Hospital/Long Island Jewish Medical Center

Presenting Author Manile A. Dastagir

Track Adult

Keywords

X-linked agammaglobulinemia

sinusoidal obstruction syndrome

noncirrhotic portal hypertension

cholestatic liver dysfunction

portal hypertension

jaundice

pancytopenia

IVIG

hepatic pathology

liver injury

Sinusoidal Obstruction

Non-cirrhotic Portal Hypertension

Abdominal Pain

Jaundice

Hyperbilirubinemia