P0713 / #1204: HEPATIC SINUSOIDAL OBSTRUCTION SYNDROME AS A CAUSE OF MULTIORGAN DYSFUNCTION IN TWO PEDIATRIC ONCOLOGY PATIENTS WITH FIRST-LINE CHEMOTHERAPY

Aims & Objectives: Hepatic Sinusoidal obstruction syndrome (SOS/VOD) is a toxic complication common after stem cell transplant (HSCT). It may also occur after chemotherapy without HSCT. We present two cases who developed severe SOS/VOD with multiorgan dysfunction syndrome (MODS) after chemotherapy. Methods: Retrospective case and literature review. Results: Patient 1 was 3-month-old male with infantile fibrosarcoma. He received actinomycin and vincristine two days prior. He was admitted to PICU for sepsis quickly developing hepatic-predominant (Total Bilirubin 4.6 TGO 6457 TGP 1838, ascites) MODS. Infectious etiologis including bacteremia, CNS infection and cytomegalovirus were excluded. Doppler ultrasound revealed portal hypertension with reverse flow. Biopsy showed sinusoidal dilation and congestion. Patient received methylprednisolone pulses (500mgm2do every q12, 6 doses). Hepatic dysfunction improved but course was complicated with Candidemia and intestinal perforation. Patient ultimately recovered and was discharged from PICU after 72 days. Patient 2 was 17-year-old male with metastatic testicular germ-cell tumor. He received bleomycin, carboplatin and etoposide 5 days prior. He was admitted to PICU for shock and developed hepatic-predominant (total bilirubin 9, direct 7.1, ascites) MODS. Infectious causes were excluded. Doppler ultrasound revealed portal hypertension and reverse flow. He received methylprednisolone pulses (500mgm2do every 12 hours) interrupted at 3 doses because of developing pancreatitis, with fluconazole prophylaxis. Patient recovered and was discharged from PICU after 21 days Conclusions: SOS/VOD is a rare but severe cause of MODS in children with cancer. In cases where defibrotide is not available, methylprednisolone may be a therapeutic option albeit with important adverse events.