Sakurai Tamaki, Kuroda Hiroyuki, Yamada Michiko, Arihara Yohei, Jyomen Wataru, Hirako Tasuku, Abe Tomoyuki, Fujii Shigeyuki, Maeda Masahiro, Fujita Miri, Kato Junji
Department of Gastroenterology and Hematology/Clinical Oncology, Internal Medicine, Steel Memorial Muroran General Hospital.
Rinsho Ketsueki. 2013 Jun;54(6):574-8.
We describe a rare case of acute promyelocytic leukemia (APL) presenting with central nervous system (CNS) involvement at the time of initial diagnosis. A 58-year-old male was hospitalized with palpitations, dyspnea, high grade fever, photophobia, and disturbance of consciousness in March 2010. APL was diagnosed by bone marrow (BM) examination. The cytogenetic analysis of BM cells demonstrated t(15;17)(q22;q11), and PML-RARA chimeric gene was detected by reverse transcriptase-polymerase chain reaction assay. Magnetic resonance imaging of the brain revealed several high intensity regions in the cerebrum and cerebellum. CNS involvement was diagnosed based on the appearance of APL blasts in cerebrospinal fluid (CSF). The patient was treated with all-trans retinoic acid (ATRA), and systemic chemotherapy consisting of idarubicin and cytarabine according to the Japan Adult Leukemia Study Group (JALSG) APL 204 protocol. He was then treated with continuous intrathecal administration of cytotoxic drugs (methotrexate, cytarabine, prednisolone) after systemic chemotherapy, achieving complete remission (CR) in both BM and the CNS. To date, he has been maintained in complete molecular remission in both BM and the CSF for 28 months, to date.
我们描述了一例罕见的急性早幼粒细胞白血病（APL），在初诊时即出现中枢神经系统（CNS）受累。一名58岁男性于2010年3月因心悸、呼吸困难、高热、畏光及意识障碍入院。通过骨髓（BM）检查诊断为APL。对BM细胞进行细胞遗传学分析显示t(15;17)(q22;q11)，通过逆转录聚合酶链反应检测到PML-RARA嵌合基因。脑部磁共振成像显示大脑和小脑有多个高强度区域。根据脑脊液（CSF）中出现APL原始细胞诊断为CNS受累。患者接受了全反式维甲酸（ATRA）治疗，并根据日本成人白血病研究组（JALSG）APL 204方案接受了由伊达比星和阿糖胞苷组成的全身化疗。在全身化疗后，他又接受了连续鞘内注射细胞毒性药物（甲氨蝶呤、阿糖胞苷、泼尼松龙）治疗，在BM和CNS均实现完全缓解（CR）。迄今为止，他的BM和CSF已维持完全分子缓解状态达28个月。
