Treatment Outcomes of Langerhans Cell Histiocytosis: A Retrospective Study

• Published in: Medicina (Kaunas, Lithuania) Vol. 57; no. 4; p. 356
• Main Authors: Hashimoto, Kazuhiko, Nishimura, Shunji, Sakata, Naoki, Inoue, Masami, Sawada, Akihisa, Akagi, Masao
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 07.04.2021; MDPI
• Subjects: Antigens; Autoimmune diseases; Biopsy; Bone marrow; Chemotherapy; Female; histiocytosis; Histiocytosis, Langerhans-Cell - diagnostic imaging; Histiocytosis, Langerhans-Cell - drug therapy; Humans; Kinases; Langerhans cells; Lymphatic system; Magnetic resonance imaging; Male; Medical prognosis; outcomes; Pain; Pathogenesis; Patients; Pediatrics; Positron Emission Tomography Computed Tomography; Prostate; Rare diseases; Retrospective Studies; Scintigraphy; Skin; Spleen; Tomography, X-Ray Computed; Treatment Outcome; Vertebrae; Langerhans cells; outcomes; histiocytosis
• ISSN: 1648-9144; 1010-660X; 1648-9144
• DOI: 10.3390/medicina57040356

: Langerhans cell histiocytosis (LCH) is a rare disease characterized by the infiltration of one or more organs by Langerhans cell-like dendritic cells. LCH often involves the bone, and its clinical evidence is limited. The purpose of this study is to report on the treatment of LCH at our institution and to add to the evidence for LCH. : We reviewed six cases of LCH treated in our hospital between November 2005 and February 2016. Patient age at the first visit, sex, site of origin, symptoms, image tools used for diagnosis, biopsy site, complications, treatment, and final clinical outcome were evaluated. The median follow-up period was 41 months. : The median patient age at the first visit was 13.5 years. Three male and three female individuals were enrolled. Multiple lesions were observed in five cases, and a solitary lesion was observed in one case. Pain was the chief complaint in five cases. Radiography was the most commonly used imaging tool. Bone scintigraphy or magnetic resonance imaging and positron emission tomography-computed tomography were also used to diagnose systematic LCH. Biopsy of the femur was performed in two cases, and biopsy of the tibia, lumbar vertebrae, rib, and radius was performed in one case each. Regarding comorbidities, one case of hepatitis B and one case of autism were observed. Chemotherapy was initiated in two patients. The other four patients were observed naturally. Continuous disease-free survival was observed in five patients. One patient remained alive but not without disease during the final follow-up examination. : LCH should be diagnosed as early as possible to treat it appropriately.