Ultrasonographic scoring system for SOS/VOD in pediatric hematopoietic stem cell transplant recipients

Sinusoidal obstruction syndrome (SOS)/veno-occlusive disease (VOD) is a serious complication following hematopoietic stem cell transplantation. It is more common in children, with an incidence of approximately 20% compared to 10% in adult patients. While the Hokkaido ultrasonography (US)-based scoring system (HokUS-10 and HokUS-6) has proven effective in diagnosing SOS/VOD in adults, its utility in children remains uncertain. This study evaluated the utility of the HokUS-10/6 scoring systems in diagnosing pediatric SOS/VOD (pSOS/VOD) in a cohort of 99 patients, including 13 diagnosed with pSOS/VOD. Hyperbilirubinemia (> 2 mg/dL) was observed in only three patients (23%), whereas transfusion-refractory thrombocytopenia, hepatomegaly, and ascites were observed in all 13 patients. When the cutoff values for HokUS-10 and HokUS-6 scores were set at 4 and 2, the respective areas under the curve were 0.977 and 0.957. The sensitivity and specificity were 100% and 80% for HokUS-10, and 77% and 97% for HokUS-6, respectively. The simplified HokUS-6 may be a practical tool for diagnosing pSOS/VOD when use of the HokUS-10 scoring system is not feasible. HokUS-10/6 is important for early detection of pSOS/VOD during regular examinations and plays a valuable role in the diagnosis of clinical pSOS/VOD.