Minami Mariko, Shima Takahiro, Kato Koji, Yamamoto Hidetaka, Tsuchihashi Kenji, Oku Seido, Shimokawa Tomonori, Tochigi Taro, Yoshimoto Goichi, Kamezaki Kenjiro, Takenaka Katsuto, Iwasaki Hiromi, Oda Yoshinao, Miyamoto Toshihiro, Akashi Koichi
Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Science, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
Int J Hematol. 2015 Aug;102(2):244-8. doi: 10.1007/s12185-015-1778-0. Epub 2015 Mar 10.
Langerhans cell histiocytosis (LCH) is a rare disease in adults. The treatment strategy for this condition remains controversial. Intensified systemic chemotherapy is required in pediatric patients with the multiple system form of LCH (MS-LCH) for aggressive forms of the disease. Recent clinical trials have shown that intensified chemotherapy for pediatric patients diagnosed with MS-LCH results in improved outcomes. However, whether the feasibility and efficacy of an intensified systemic chemotherapy regimen are also beneficial for adult patients with MS-LCH remains unclear. Here, we report two cases of adult MS-LCH that were successfully treated with an intensified treatment protocol as used in pediatric patients. One patient fully completed the protocol, and has since maintained a complete response (CR) for 2 years following completion of the treatment. The other patient also achieved CR after induction therapy, and is now undergoing maintenance therapy in an outpatient clinic. The cases presented in this study suggest that intensified systemic chemotherapy as used for pediatric patients with MS-LCH is well tolerated and effective for adult patients as well.
朗格汉斯细胞组织细胞增多症（LCH）在成人中是一种罕见疾病。针对这种病症的治疗策略仍存在争议。对于患有多系统形式的LCH（MS-LCH）且病情呈侵袭性的儿科患者，需要强化全身化疗。最近的临床试验表明，对诊断为MS-LCH的儿科患者进行强化化疗可改善预后。然而，强化全身化疗方案的可行性和有效性对成年MS-LCH患者是否也有益尚不清楚。在此，我们报告两例成年MS-LCH患者，他们采用儿科患者使用的强化治疗方案成功治愈。一名患者完全完成了该方案，治疗完成后已维持完全缓解（CR）状态达2年。另一名患者在诱导治疗后也实现了CR，目前正在门诊接受维持治疗。本研究中呈现的病例表明，用于儿科MS-LCH患者的强化全身化疗对成年患者也具有良好的耐受性且有效。
