Prognosis of pediatric patients with anicteric and late-onset sinusoidal obstruction syndrome after hematopoietic stem cell transplantation.

BACKGROUND

Sinusoidal obstruction syndrome (SOS) is a life-threatening complication after hematopoietic stem cell transplantation (HSCT). Most adult patients with SOS present with jaundice, whereas hyperbilirubinemia does not always occur in children. Additionally, while late-onset SOS is rare in adults, 15-20% of SOS cases develop beyond day 30 after HSCT in children.

PROCEDURE: We investigated the incidence and prognosis of children with anicteric and late-onset SOS. We retrospectively analyzed the data of patients who developed SOS after HSCT conducted at our center between 2000 and 2016.

RESULTS

A total of 22 patients with a median age of 6.5 years (range: 0-16), including 14 males and eight females, developed SOS. Eight of the twenty-two patients were diagnosed as having anicteric SOS, and nine as having late-onset SOS. Patients with anicteric SOS had significantly lower incidence of SOS-related death at 100 days after HSCT (12.5% vs 64.3%, P = 0.03) and higher 2-year overall survival (OS) rate (60.0% vs 14.3%, P = .04) than patients with icteric SOS. One patient with anicteric SOS died from progression of SOS. There were no significant differences observed in these endpoints between patients who developed SOS before and after 21 days from HSCT.

CONCLUSIONS

Careful monitoring is needed for the development of SOS even in the absence of jaundice, and even at 3 weeksafter HSCT in children.