Haematopoietic stem-cell transplantation: Case

Framing the case

This six-year-old boy is on day fourteen of an allogeneic matched unrelated donor transplant, with the weight gain, the tender hepatomegaly and the rising bilirubin that together point to the hepatic sinusoidal obstruction syndrome. The timing, within the first twenty-one days of the myeloablative conditioning, the total body irradiation and the etoposide, is the classic window for the syndrome, and the weight gain of eight percent from the baseline alongside the tender liver and the jaundice meets the diagnostic triad. The framework is the recognition that this is a hepatotoxic complication of the conditioning, distinct from the graft-versus-host disease and the drug injury, and that the early recognition and the prompt defibrotide are the decisive interventions. ^[8]

The differential and the diagnostic pathway

The hepatic differential at this point is the sinusoidal obstruction syndrome against the hepatic graft-versus-host disease, the drug-induced liver injury from the antifungal or the antibiotic, and the viral hepatitis. The sinusoidal obstruction syndrome presents early, within the first twenty-one days, with the weight gain and the tender liver, while the hepatic graft-versus-host presents later, often after the engraftment, with the jaundice and the gut involvement. The diagnosis of the sinusoidal obstruction syndrome is clinical, supported by the ultrasound with the doppler showing the reversal of the portal flow and the elevated hepatic pressures, with the transjugular liver biopsy and the pressure measurement in the selected case. The viral studies exclude the cytomegalovirus and the hepatitis, and the drug history excludes the reaction. ^[8][1]