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Philadelphia chromosome‐positive acute lymphoblastic leukemia and Down syndrome
Author(s) -
Tamaura Moe,
Iwasaki Fuminori,
Yokosuka Tomoko,
Fukuda Kunio,
Hamonoue Satoshi,
Goto Hiroaki
Publication year - 2016
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.12978
Subject(s) - medicine , imatinib , lymphoblastic leukemia , chemotherapy , philadelphia chromosome , hematopoietic stem cell transplantation , sibling , pediatrics , down syndrome , transplantation , oncology , leukemia , chromosomal translocation , genetics , myeloid leukemia , gene , psychology , developmental psychology , psychiatry , biology
Abstract Children with Down syndrome (DS) are at high risk of developing acute lymphoblastic leukemia (ALL), but Philadelphia chromosome‐positive (Ph+) ALL is rare in DS children. We report a case of Ph+ALL with DS complicated by chronic heart failure. Complete molecular remission was obtained after imatinib‐combined chemotherapy, although infectious episodes during the neutropenic period worsened the heart condition. After two courses of intensification chemotherapy, the patient underwent reduced intensity stem cell transplantation from an HLA‐identical sibling donor followed by post‐transplant imatinib. The patient maintained molecular complete remission for >2 years. This case report is the first description of the safe and effective use of imatinib for DS‐Ph+ALL. This case suggests the potential of molecular targeting therapy in DS‐ALL, which is often complicated by congenital diseases, and the importance of treating DS‐ALL while maintaining intensity and reducing treatment‐related toxicity.