Kishimoto S, Ishii E, Murakami Y, Takeshita M, Watanabe M, Sakai R, Miyazaki S
Division of Pediatrics, Saga Prefectural Hospital Koseikan, Japan.
Pediatr Hematol Oncol. 1997 Mar-Apr;14(2):169-75. doi: 10.3109/08880019709030903.
We describe here the first case of childhood acute promyelocytic leukemia (APL) with cutaneous infiltration of leukemic cells following treatment with all-trans retinoic acid (ATRA) confirmed by immunostaining and polymerase chain reaction for PML/RAR alpha. An 11-year-old girl was diagnosed as having APL. Chromosomal analysis demonstrated the characteristic karyotype of t(15;17). ATRA therapy was begun at a dose of 45 mg/m2 daily. During ATRA therapy, leukocytosis and retinoic acid syndrome were observed but were resolved by dexamethasone. Two months after commencement of ATRA therapy, complete remission was achieved. During the course of consolidation chemotherapy, however, multiple cutaneous nodules developed in her trunk, the size and number of which increased despite intensive chemotherapy. Histological and immunological studies of the cutaneous nodules showed infiltration of leukemic cells. PML/RAR alpha mRNA was detected in both the cutaneous nodules and bone marrow by means of polymerase chain reaction. ATRA treatment for APL may be associated with an increased incidence of extramedullary disease such as cutaneous lesions. The best available therapy for APL may be a combination of ATRA and chemotherapy, especially when a marked leukocytosis occurs during ATRA therapy.
我们在此描述首例儿童急性早幼粒细胞白血病（APL），经免疫染色和PML/RARα聚合酶链反应证实，在全反式维甲酸（ATRA）治疗后出现白血病细胞皮肤浸润。一名11岁女孩被诊断为APL。染色体分析显示特征性核型t(15;17)。开始以每日45 mg/m²的剂量进行ATRA治疗。在ATRA治疗期间，观察到白细胞增多和维甲酸综合征，但用地塞米松后症状缓解。ATRA治疗开始两个月后实现完全缓解。然而，在巩固化疗过程中，她的躯干出现多个皮肤结节，尽管进行了强化化疗，其大小和数量仍增加。对皮肤结节进行组织学和免疫学研究显示有白血病细胞浸润。通过聚合酶链反应在皮肤结节和骨髓中均检测到PML/RARα mRNA。ATRA治疗APL可能与髓外疾病如皮肤病变的发生率增加有关。APL的最佳可用治疗方法可能是ATRA与化疗联合，尤其是在ATRA治疗期间出现明显白细胞增多时。
