Fig 1.
The pathophysiology of sinusoidal obstruction syndrome.
The presumed pathophysiological mechanism underlying sinusoidal obstruction syndrome is a drug-mediated damage to sinusoidal endothelial cells, causing swelling and loss of fenestration. This allows red blood cells to enter the space of Disse and dissect off the endothelial lining. The sloughed off cells embolise downstream and cause obstruction of the hepatic microcirculation and consequently hepatocellular necrosis. [7] These pathophysiological changes lead to the clinical symptoms: jaundice, tender hepatomegaly, ascites and fluid retention. Figure by Linea Natalie Toksvang.
Fig 2.
PRISMA flow diagram of study selection.
Table 1.
Overview of GRADE evaluation.
Fig 3.
Incidence of sinusoidal obstruction syndrome (SOS) or nodular regenerative hyperplasia (NRH) compared to 6-thioguanine (6TG) dose in the included studies.
Studies with reported mean or median 6TG dose and incidence of hepatotoxicity defined as either SOS or NRH are depicted. Doses in mg/m2 were calculated with the assumption that an adult is 1.73 m2, and that 30 kg correspond to one m2. The dotted line refers to an incidence of 6%, corresponding to the suggested background incidence.