Clinical Case Summary
Hematopoietic stem cell transplantation (HPSCT) is associated with multiple complications, such as hepatic sinusoidal obstruction syndrome (SOS) (hepatomegaly, ascites, jaundice, and thrombocytopenia) and graft-versus-host disease (GVHD) (skin, gastrointestinal tract and liver are the main targets). These entities may present overlapping clinical findings, being considered differential diagnoses. Coexistence is rare.
A 29-year-old man undergoes HPSCT for acute myeloid leukemia. On day(D)+20, he presents with hyperbilirubinemia (HBR), pleural effusion, ascites, painful hepatomegaly. Ultrasound is suggestive of SOS – Defibrotide was initiated. On D+44, acute cutaneous, intestinal and hepatic GVHD develops. He started methylprednisolone, with analytical improvement. On D+132, worsening cholestasis and abdominal pain. MRCP revealed caliber compromise in several segments of the intrahepatic bile ducts and wall irregularity of the main bile duct. Due to clinical and analytical worsening, he was admitted to the ICU. As he maintained total HBR (30mg/dL) and thrombocytopenia (30,000cell/uL), he underwent an hepatic hemodynamic study (hepatic venous pressure gradient of 10 mmHg) and a transjugular liver biopsy that showed canalicular hepatic cholestasis, bile duct injury and focal hepatocellular necrosis - probable GVHD – and injury to centrilobular veins and centrilobular necrosis - possible SOS. Mycophenolate mofetil was started, but on D+195 the patient died.
This case is notable for its complexity and for demonstrating the rare co-existence of histological aspects of SOS (several weeks after its diagnosis and clinical resolution) and GVHD. Although the clinical and analytical findings may be sufficient for the diagnosis, it is important to highlight the importance of transjugular liver biopsy in patients with severe thrombocytopenia for the characterization and histological confirmation of cholestatic hepatitis, especially when the etiology may be multifactorial.
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