The Japanese Journal of Pediatric Hematology
Online ISSN : 1884-4723
Print ISSN : 0913-8706
ISSN-L : 0913-8706
Clinical Course of Six Patients with Childhood Acute Lymphoblastic Leukemia and Down Syndrome
Kazuko KUDOMasaaki YANAINaoko MAEDAKoji KATOKeizo HORIBETakaharu MATSUYAMASeiji KOJIMA
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2001 Volume 15 Issue 6 Pages 451-456

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Abstract

We treated six patients with childhood acute lymphoblastic leukemia (ALL) and Down syndrome (DS) during the period from 1987 to 1997. The median age at diagnosis was three years and eight months. The median white blood cell count was16, 800/μl. The analysis of immunophenotypes of the blasts revealed that five had pre-B cell type and one had biphenotypic ALL. Three were treated by Tokai Pediatric Oncology protocol, one by JACLS-ALL 97 protocol and the other two by multidrug combination therapy, including CHOP. Although all six patients had achieved complete remission, two relapsed. One patient died of serious bacterial infection after relapse, and one received autologous bone marrow transplantation with a preconditioning regimen using L-PAM and TBI, resulting in long-term survival. Three patients remain in the first remission. One died of liver dysfunction because of methotrexate (MTX) during maintenance therapy with no evidence of recurrence. Plasma concentrations after high-dose MTX (3g/m2) therapy were higher in patients with DS than in patients without it, but there was no statistical significance. The concentrations that followed after reduced MTX (1-2 g/m2) therapy were almost equal among those with DS and those with MTX (3g/m2) without DS.

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