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急性间歇性卟啉症患者的急性髓细胞白血病多药化疗

Polychemotherapy of acute myelogenous leukemia in a patient with acute intermittent porphyria.

作者信息

Wehmeier A, Fischer J T, Goerz G, Schneider W

出版信息

Klin Wochenschr. 1987 Apr 1;65(7):338-40. doi: 10.1007/BF01745391.

Abstract

The safety of drugs in hepatic porphyrias has largely been established by clinical experience, which is very limited in the case of antineoplastic agents. We administered three cycles of polychemotherapy consisting of daunorubicin, cytarabine and 6-thioguanine, and modified supportive care to a 33-year-old Turkish woman suffering from acute myelogenous leukemia. The urinary excretion of total porphyrins, porphobilinogen, and aminolevulinic acid was continuously monitored. Excretion of these metabolites was permanently elevated, but the values were comparatively low during cytotoxic therapy while peak values were recorded at the onset of fever during bone marrow aplasia; yet there were no clinical signs of porphyritic attacks at that time. A few potentially unsafe drugs were tolerated without an increase in porphyrin excretion. Although the susceptibility to drugs is highly variable in patients with hepatic porphyrias, the treatment of malignancy in these patients seems justified as long as porphyrin excretion under therapy is not grossly elevated over baseline values and appropriately modified supportive care is administered.

摘要

药物在肝性卟啉病中的安全性很大程度上已由临床经验确定,但在抗肿瘤药物方面,临床经验非常有限。我们对一名33岁患有急性髓性白血病的土耳其女性进行了三个周期的联合化疗,化疗方案包括柔红霉素、阿糖胞苷和6-硫鸟嘌呤,并对支持治疗进行了调整。持续监测了总卟啉、胆色素原和δ-氨基乙酰丙酸的尿排泄情况。这些代谢产物的排泄一直升高,但在细胞毒性治疗期间数值相对较低,而在骨髓再生障碍发热开始时记录到峰值;然而,当时没有卟啉病发作的临床体征。一些潜在不安全的药物在未增加卟啉排泄的情况下被耐受。尽管肝性卟啉病患者对药物的易感性差异很大,但只要治疗期间的卟啉排泄没有比基线值大幅升高,并给予适当调整的支持治疗,对这些患者进行恶性肿瘤治疗似乎是合理的。

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