Haematopoietic stem-cell transplantation: Viva

Framing the problem

This girl is eighteen days after a matched unrelated donor transplant, which places her in the engraftment period when both the acute graft-versus-host disease and the sinusoidal obstruction syndrome declare themselves. The rash on the palms and soles points toward the acute graft-versus-host disease, while the tender liver points toward the sinusoidal obstruction syndrome, and the rising bilirubin is common to both. The framework is the recognition that these two complications may coexist, that each has a specific therapy, and that the tissue diagnosis settles the question before the empiric treatment is escalated. The first priority is to resuscitate the unstable elements and to confirm the cause. ^[5]

The differential and the diagnostic pathway

The examiner will probe the differential, and the candidate must separate the graft-versus-host from the sinusoidal obstruction syndrome and from the mimics. The acute graft-versus-host disease presents with the rash, the diarrhoea and the bilirubin rise, and it is graded by the extent of the skin, the bilirubin level and the stool volume. The sinusoidal obstruction syndrome presents with the weight gain, the tender hepatomegaly and the jaundice within the first twenty-one days, and it is supported by the ultrasound with the reversal of the portal flow. The drug eruption and the viral hepatitis add to the differential. The skin and the liver biopsy settle the graft-versus-host, and the ultrasound and the hepatic pressures settle the sinusoidal obstruction syndrome. ^[8][5]

The management of the acute graft-versus-host disease