METHODS: We report a 10-year-old male on maintenance chemotherapy for pre-Bcell ALL who presented to the hospital with worsening oral lesions and erythematous, fissured plaques on the palms and soles. Maintenance therapy consisted of IV vincristine and 5-day pulse of steroids every 12 weeks, daily 6MP, and weekly MTX, which were increased to ≥ 150% of standard dosing due to persistent absolute neutrophil counts > 1500. Metabolites obtained on admission demonstrated elevated 6MMP metabolites at 35,761 (normal < 5700). TPMT and NUDT15 enzyme activity were normal and no alterations in genotyping were discovered.
RESULTS: Patient\'s oral chemotherapy, including both 6MP and MTX, were stopped and allopurinol 100 mg daily was initiated, which lead to overall improvement.
CONCLUSIONS: Clinical findings of acute mucositis and worsening of hand-foot syndrome, in the setting of inadequate myelosuppression in a child on maintenance therapy for ALL should raise concerns to consider altered metabolism pathway leading to toxic metabolite buildup. Allopurinol can play in improving cutaneous manifestation and chemotherapeutic dosing in patients with altered metabolism.
方法:我们报告了一名10岁的男性,正在接受前BcellALL的维持化疗,他出现口腔病变恶化和红斑,手掌和脚底上的裂开的斑块。维持治疗包括静脉注射长春新碱和5天脉冲类固醇每12周,每天6MP,每周MTX,由于持续的中性粒细胞绝对计数>1500,增加到标准给药的≥150%。入院时获得的代谢物显示6MMP代谢物升高,为35,761(正常<5700)。TPMT和NUDT15酶活性正常,未发现基因分型改变。
结果:患者口服化疗,包括6MP和MTX,停止并开始每天100毫克别嘌呤醇,这导致整体改善。
结论:急性粘膜炎和手足综合征恶化的临床表现,在接受ALL维持治疗的儿童骨髓抑制不足的情况下,人们应该担心代谢途径的改变会导致毒性代谢物的积累.别嘌呤醇可以改善代谢改变的患者的皮肤表现和化疗剂量。