İnce Dilek, Demirağ Bengü, Özek Gülcihan, Erbay Ayşe, Ortaç Ragıp, Oymak Yeşim, Kamer Serra, Yaman Yöntem, Kundak Selcen, Vergin Canan
Department of Pediatric Oncology, Dokuz Eylül University Institute of Oncology, İzmir.
Department of Pediatric Hematology-Oncology Clinic, Dokuz Eylül University Institute of Oncology, İzmir.
Turk J Pediatr. 2016;58(4):349-355. doi: 10.24953/turkjped.2016.04.001.
This study aimed to analyze children with the diagnosis of Langerhans cell histiocytosis (LCH) who were diagnosed and treated between 1998-2015. Medical records were evaluated retrospectively for clinical and laboratory features, treatment details, and outcome. There were 20 patients, the median age of diagnosis was 37 months, M/F ratio: 1.5. Nine had single system (SS), 11 had multisystem (MS) LCH. Spontaneous regression occurred in three infants with skin limited LCH. Eight patients had risk organ involvement in MS-LCH group. The curettage alone was performed in only one case. Patients received LCH-II/ LCH-III based chemotherapy schema. Radiotherapy was performed to vertebral disease and residual craniofacial bone disease in four cases. The regression and relapse rates were 100% and 33% for SS-LCH. The regression and relapse rates were 73%, and 18% for MS-LCH. Two infants with MS-LCH died despite chemotherapy. Pulmonary and liver involvements affected outcome adversely in MS-LCH. Multidisciplinary treatment approaches are needed.
本研究旨在分析1998年至2015年间确诊并接受治疗的朗格汉斯细胞组织细胞增多症（LCH）患儿。对病历进行回顾性评估，以了解临床和实验室特征、治疗细节及结果。共有20例患者，诊断时的中位年龄为37个月，男/女比例为1.5。9例为单系统（SS）LCH，11例为多系统（MS）LCH。3例皮肤局限性LCH婴儿出现自发消退。MS-LCH组有8例患者出现风险器官受累。仅1例患者接受了单纯刮除术。患者接受基于LCH-II/LCH-III的化疗方案。4例患者对脊柱疾病和残留颅面骨疾病进行了放疗。SS-LCH的消退率和复发率分别为100%和33%。MS-LCH的消退率和复发率分别为73%和18%。2例MS-LCH婴儿尽管接受了化疗仍死亡。肺部和肝脏受累对MS-LCH的预后产生了不利影响。需要多学科治疗方法。
