Sinusoidal Obstruction Syndrome Post-allogeneic Transplantation: A Complex Multisystem Challenge

Area of Science:

• Hematology
• Transplantation immunology
• Critical care medicine

Background:

• Haploidentical allogeneic stem cell transplantation is a potentially curative therapy for chronic myeloid leukemia (CML) in blast crisis.
• Post-transplant complications, including sinusoidal obstruction syndrome (SOS), can significantly impact patient outcomes.
• Managing severe SOS requires a multidisciplinary approach and careful consideration of therapeutic options.

Observation:

• A 41-year-old female with CML in blast crisis developed severe sinusoidal obstruction syndrome (SOS) on post-transplant day 10 following haploidentical allogeneic stem cell transplantation.
• Clinical manifestations included hyperbilirubinemia, hepatosplenomegaly, fluid retention, and portal vein dilation.
• The patient experienced complications such as neutropenic sepsis, mucositis, thrombocytopenia, gastrointestinal hemorrhage, hepatorenal syndrome requiring CRRT, and acute hypoxic respiratory failure.

Findings:

• Defibrotide administration showed a therapeutic response with declining bilirubin levels but required dose interruptions due to thrombocytopenia and hemorrhage.
• Despite intensive supportive care, including fluid management, albumin, diuresis, and CRRT, the patient progressed to multiorgan failure.
• The case underscores the challenges in diagnosing and treating severe post-transplant SOS.

Implications:

• Early recognition and intervention are critical for improving outcomes in patients with severe post-transplant SOS.
• A careful risk-benefit assessment of defibrotide is necessary, considering potential adverse effects.
• Multidisciplinary critical care strategies are essential for managing high-risk patients with complex post-transplant complications.