Egeler R M, de Kraker J, Voûte P A
Emma Kinderziekenhuis/Kinder AMC, Academical Medical Centre, Department of Pediatric Oncology, Amsterdam, The Netherlands.
Med Pediatr Oncol. 1993;21(4):265-70. doi: 10.1002/mpo.2950210406.
Fifty-two pediatric patients with Langerhans cell histiocytosis (LCH) were diagnosed at the Emma Kinderziekenhuis (EKZ) in Amsterdam over a 20-year period. Eight patients with multiorgan involvement with organ dysfunction and ten patients with multi-organ involvement without organ dysfunction received chemotherapy containing cytosine-arabinoside, vincristine, and prednisolone as part of their treatment. Five of the 8 patients (63%) with organ dysfunction and eight of the 10 (80%) without the organ dysfunction who needed chemotherapy because of the deteriorating of symptoms despite conventional therapy are presently in complete clinical remission. Two of those with organ dysfunction have died. Four of the total 18 patients developed diabetes insipidus either as an initial symptom or during the course of the disease. These results in both groups compare satisfactorily with other chemotherapeutic regimens, and since this combination is only mildly toxic, it has been well tolerated. Therefore we would recommend wider experience using this regimen in patients with widespread LCH with organ dysfunction as well as in patients with disseminated LCH in which chemotherapy proved to be necessary.
在20年的时间里，阿姆斯特丹的艾玛儿童医院（EKZ）诊断出52例患有朗格汉斯细胞组织细胞增多症（LCH）的儿科患者。8例多器官受累且伴有器官功能障碍的患者以及10例多器官受累但无器官功能障碍的患者接受了含有阿糖胞苷、长春新碱和泼尼松龙的化疗，作为其治疗的一部分。8例有器官功能障碍的患者中有5例（63%），10例无器官功能障碍的患者中有8例（80%），尽管接受了传统治疗，但由于症状恶化而需要化疗，目前处于完全临床缓解状态。其中2例有器官功能障碍的患者已经死亡。18例患者中有4例在疾病初期或病程中出现了尿崩症。这两组的结果与其他化疗方案相比令人满意，并且由于这种联合用药毒性较小，耐受性良好。因此，我们建议在患有广泛LCH且伴有器官功能障碍的患者以及化疗被证明是必要的播散性LCH患者中更广泛地使用这种方案。
