Clin Pediatr Hematol Oncol.  2012 Oct;19(2):109-113.

6-Thioguanine-Induced Hepatic Sinusoidal Obstruction Syndrome in a Leukemic Child with TPMT Heterozygote

Affiliations
  • 1Center for Pediatric Oncology, National Cancer Center, Goyang, Korea. hjpark@ncc.re.kr
  • 2Department of Pediatrics, Inje University of Medicine, Pusan, Korea.
  • 3Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea.
  • 4Department of Radiology, College of Medicine, The Catholic University of Korea, Seoul, Korea.
  • 5Department of Laboratory Medicine, National Cancer Center, Goyang, Korea.

Abstract

Hepatic sinusoidal obstruction syndrome (hSOS) can be developed as a common complication after hematopoietic stem cell transplantation (HSCT), and rarely after 6-thioguanine-based chemotherapy without HSCT. A four-year-old boy with heterozygotic polymorphism for thiopurine methyltransferase (TPMT) developed hSOS after he received chemotherapy with cytarabine, cyclophosphamide, intrathecal methotrexate and 6-thioguanine (6-TG) as reconsolidation chemotherapy of acute lymphoblastic leukemia (ALL). He was treated with defibrotide, N-acetylcysteine, urusodeoxycholic acid, glutathione, and supportive care. He recovered completely in nine days without long-term complication, and completed chemotherapy with 6-mercaptopurine without severe complication. We report a case of hSOS developed in an ALL patient with TPMT heterozygote after 6-TG based reconsolidation chemotherapy.

Keyword

Hepatic sinusoidal obstruction syndrome; Thiopurine methyltransferase; 6-Thioguanine; Polymorphism

MeSH Terms

6-Mercaptopurine
Acetylcysteine
Child
Cyclophosphamide
Cytarabine
Glutathione
Hematopoietic Stem Cell Transplantation
Hepatic Veno-Occlusive Disease
Heterozygote
Humans
Methotrexate
Methyltransferases
Polydeoxyribonucleotides
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Thioguanine
6-Mercaptopurine
Acetylcysteine
Cyclophosphamide
Cytarabine
Glutathione
Methotrexate
Methyltransferases
Polydeoxyribonucleotides
Thioguanine
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