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Veno-Occlusive Disease: A Life-saving Novel Approach With Plasma Exchange, IVIG, and Steroid, Without Defibrotide
Banu Katlan1, Funda Erkasar2, Mesut Topdemir3
1Department of Pediatrics, Division of Pediatric Intensive Care Unit.
Insights
Severe hepatic veno-occlusive disease (VOD) in acute lymphoblastic leukemia (ALL) patients can be rapidly treated with therapeutic plasma exchange (TPE), intravenous immunoglobulin (IVIG), and methylprednisolone (MPZ). This combination offers an alternative to Defibrotide.
Area of Science:
- Hematology
- Oncology
- Hepatology
Background:
- Hepatic veno-occlusive disease (VOD) is a life-threatening complication of hematopoietic stem cell transplantation and chemotherapy.
- Defibrotide is the sole approved agent for VOD, but alternative treatments are needed.
- Acute lymphoblastic leukemia (ALL) patients undergoing treatment are at risk for VOD.
Observation:
- A case of severe VOD in an ALL patient is presented.
- The patient received a combination of therapeutic plasma exchange (TPE), intravenous immunoglobulin (IVIG), and methylprednisolone (MPZ).
Findings:
- The combined therapeutic approach led to rapid and significant clinical and laboratory improvement.
- This treatment strategy demonstrated effectiveness in managing severe VOD.
Implications:
- Therapeutic plasma exchange (TPE), IVIG, and MPZ represent a viable alternative treatment for severe VOD in ALL patients.
- These findings offer crucial insights for managing VOD when Defibrotide is unavailable.
- This approach may improve outcomes for VOD patients in resource-limited settings.
Introduction:
Hepatic veno-occlusive disease (VOD) is a critical medical emergency with a high mortality rate of up to 90% if not promptly treated. Defibrotide is the only approved medication for VOD treatment, exhibiting anti-inflammatory, antithrombotic, and anti-ischemic properties. This report presents a case of severe VOD in a patient undergoing acute lymphoblastic leukemia (ALL) treatment.
Case Presentation:
We describe the successful and rapid treatment of severe VOD in an ALL patient using therapeutic plasma exchange (TPE), intravenous immunoglobulin (IVIG), and methylprednisolone (MPZ). The patient showed significant clinical and laboratory improvement after this combined therapeutic approach.
Conclusion:
This case highlights the effectiveness of TPE, IVIG, and MPZ in the treatment of severe VOD in ALL patients, providing insights into alternative therapeutic strategies in the absence of Defibrotide.
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