A 32-year-old woman develops tense ascites, tender hepatomegaly, and abdominal pain over three weeks. Doppler ultrasound shows absent flow in the major hepatic veins with caudate lobe hypertrophy. Bone marrow evaluation is most likely to reveal:

• A Polycythemia vera with JAK2 V617F mutation ✓
• B Acute lymphoblastic leukemia
• C Refractory anemia with ring sideroblasts
• D Hairy cell leukemia

Explanation

This is Budd-Chiari syndrome, thrombosis of the hepatic veins or suprahepatic inferior vena cava presenting with the triad of abdominal pain, hepatomegaly, and ascites. Myeloproliferative neoplasms, especially polycythemia vera, are the leading cause worldwide, identified by the JAK2 V617F mutation even when counts are normal. The caudate lobe hypertrophies because its independent venous drainage often remains patent.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.