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Successful Liver Transplantation for Transient Abnormal Myelopoiesis-Associated Liver Failure
Kazuaki Yasuoka1, Hirosuke Inoue, Koichi Tanaka
1Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Insights
Infants with Down syndrome (DS) face risks from transient abnormal myelopoiesis (TAM). This case report highlights liver transplantation as a potential life-saving treatment for TAM-related liver failure in DS infants.
Area of Science:
- Hematology
- Hepatology
- Pediatrics
Background:
- Infants with Down syndrome (DS) are susceptible to transient abnormal myelopoiesis (TAM).
- TAM can lead to fatal liver fibrosis, with limited established treatments.
- Current management for TAM-induced liver disease is primarily supportive.
Observation:
- A case of a DS infant with TAM who developed end-stage liver failure is presented.
- Liver dysfunction persisted despite the resolution of circulating blast cells.
- The infant underwent a successful living-donor liver transplantation at 56 days of life.
Findings:
- The explanted liver showed severe atrophy and fibrosis, without evidence of leukemic cell infiltration.
- The post-transplant period was favorable, with no hematological abnormalities.
- The patient is in good health 8 months after transplantation.
Implications:
- This case suggests liver transplantation may be a viable therapeutic option for TAM-related liver failure in infants with Down syndrome.
- Further research is warranted to establish the role of liver transplantation in managing this severe complication.
- This approach offers a potential life-saving intervention for a previously untreatable condition.
Abstract:
Infants with Down syndrome (DS) are at risk of developing a transient abnormal myelopoiesis (TAM). TAM occasionally involves liver fibrosis, which can be fatal. The management of liver disease in TAM has not yet been established and is mainly supportive. We report an infant with DS and TAM who developed end-stage liver failure. Liver dysfunction progressed even after blast cells disappeared from the circulation. He underwent a living-donor liver transplantation at 56 days of life without surgical complications. The explanted liver showed atrophy and severe fibrosis without leukemic cell infiltration. The posttransplant course was favorable with no hematological abnormality. He is doing well 8 months after transplantation. To the best of our knowledge, this report is the first showing that liver transplantation might be a treatment option for TAM-related liver failure.
