A remarkable presentation of a massive Budd-Chiari syndrome

A 59-year-old female presented to the emergency department with malaise, significant weight loss, abdominal discomfort, dyspnoea and severely swollen peripheral extremities. She had a past medical history of sickle cell anaemia, a latent tuberculosis infection and a chronic hepatitis B that was treated with PEG-interferon alpha-2 in 2008. Since 2018, she was lost to follow-up. The patient was critically ill, sarcopenic and lethargic. Clinical examination revealed icteric sclerae and a markedly distended and diffusely tender abdomen and peripheral oedemas. Her vital signs included a blood pressure of 105/64 mmHg, tachycardia of 130 bpm, hypothermia of 35.6°C and a SpO2 of 100% in ambient air. Laboratory workup demonstrated a total bilirubin of 12.9 mg/dL, AST 500 U/L, ALT 218 U/L, ALP 178 U/L and GGT 126 U/L, a thrombocytopenia of 34 x10E3/ μL, 16,01 x10E3/μL leukocytes, a CRP of 94.8 mg/L and a lactate of 10,12 mmol/L. The serum creatinine was 1.04 mg/dL, eGFR 54.1 ml/min. The INR was 2.77 and there was a severe hypoalbuminemia (22 g/L). D-dimers were >20 000 ng/ml. The patient presented also with spontaneous hypoglycaemia. A multiphasic contrast-enhanced thoracic and abdominal computed tomography (CT) was performed (figure 1). Given the results of the CT, a 2D echocardiogram was urgently requested (figure 2).

Keywords: HCC, Secondary Budd-Chiari Syndrome, Hepatitis B.