Abstract:
Myeloid sarcoma (MS), a tumor consisting of myeloid blasts with or without maturation, occurs at anatomical sites other than the bone marrow. MS of the gastrointestinal tract presenting with jaundice in children is rare. We report the case of a 4-year-old boy with a 6-week history of symptoms of obstructive jaundice due to a peripancreatic mass compressing the common bile duct. Six weeks later, blasts were found in a peripheral smear prior to surgical biopsy; bone marrow evaluation and flow cytometry results led to a diagnosis of acute myeloid leukemia (AML) with MS. No further invasive testing or temporary drainage was performed. He was started on induction therapy with full therapeutic doses of cytarabine, dose reductions of etoposide, and escalating doses of daunorubicin. His liver enzymes normalized, and he completed subsequent cycles of chemotherapy with full doses. The abdominal ultrasound showed resolution of the mass after the second cycle of chemotherapy. He is currently in remission three years after completing therapy. AML-directed chemotherapy in patients with obstructive jaundice secondary to MS may be beneficial without requiring invasive testing or temporary drainage procedures. Daily follow-up is crucial for chemotherapy dose modifications. Management plans should be individualized according to the patient\'s clinical condition.
摘要:
髓样肉瘤(MS),由成熟或不成熟的骨髓母细胞组成的肿瘤,发生在骨髓以外的解剖部位。儿童出现黄疸的胃肠道MS很少见。我们报告了一个4岁男孩的病例,该男孩因胰周肿块压迫胆总管而有6周的阻塞性黄疸症状。六周后,在手术活检之前,在周围涂片中发现了母细胞;骨髓评估和流式细胞术结果导致MS诊断为急性髓细胞性白血病(AML)。没有进行进一步的侵入性测试或临时引流。他开始使用全治疗剂量的阿糖胞苷进行诱导治疗,依托泊苷的剂量减少,和不断增加的柔红霉素剂量。他的肝酶恢复正常,他完成了随后的全剂量化疗周期。腹部超声显示第二周期化疗后肿块消退。他目前在完成治疗三年后处于缓解状态。在MS继发的阻塞性黄疸患者中,AML指导的化疗可能是有益的,而无需进行侵入性测试或临时引流程序。每日随访对于化疗剂量调整至关重要。管理计划应根据患者的临床情况进行个体化。