History of Present Illness:
A man in his late 70s with a history of a-fib on Eliquis and renal cell carcinoma on Yervoy (an immune checkpoint inhibitor) is brought to the ED for confusion and fever.
Vital Signs & Physical Exam:
Vital signs are normal except for a fever of 39.5 and pulse in the 120’s with a soft BP. He appears jaundiced Physical exam is otherwise normal except for mild diffused abdominal tenderness and mild to moderate confusion. There are no focal neurologic findings and the jolt sign is negative. There is no asterixis
Initial Diagnostic Testing:
- CBC: pancytopenic with WBC 1.5, Hb 12 and Plt 45
- LFTs: AST 360, ALT 120, Bili 8.2/5.8, INR 1.9
- Imaging: CXR showed CHF v. pneumonia, a CT scan is shown below
What is the most likely diagnosis?
- A) Ascites
- B) Sinusoidal Occlusion Syndrome
- C) Cholangitis
- D) Hepatic encephalopathy
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What is the most likely diagnosis?
- A) Ascites
- B) Sinusoidal Occlusion Syndrome – CORRECT – CT below shows ascites anterior to the liver (darker line with white arrowhead) as well as behind the spleen on the bottom of the image)
- C) Cholangitis
- D) Hepatic encephalopathy
1-Minute EM Consult on the topic for this case from the Emergency Medicine 1-minute Consult Pocketbook
Hepatic Sinusoidal Occlusion Syndrome (SOS):
- Clinical: Jaundice, hepatomegaly, elevated LFTs and ascites
- Risks: Immune checkpoint inhibitor treatment, stem cell transplant, ALL
- Treatment: Steroids. Mortality is around 80% if untreated
CASE CONCLUSION: Started on antibiotics for possible pneumonia and steroids for SOS (Sinusoidal Occlusion Syndrome) and admitted. Concern for HLH (hemophagocytic lymphohistiocytosis) given concomitant pancytopenia. Likely had this as well as both ferritin and triglycerides came back quite high. Fortunately responded to steroids