Kamal Achmad Fauzi, Luthfi Andi Praja Wira Yudha
Department of Orthopaedic and Traumatology, Dr.Cipto Mangunkusumo General Hospital/Faculty of Medicine Universitas Indonesia, Jakarta, Indonesia.
Ann Med Surg (Lond). 2019 Aug 2;45:102-109. doi: 10.1016/j.amsu.2019.07.030. eCollection 2019 Sep.
Langerhans cell histiocytosis (LCH) is a rare group of disorders without a well understood etiology. Known formerly as histiocytosis X, the disease has a wide spectrum of clinical presentations, including eosinophilic granuloma (solitary bone lesion), diabetes insipidus, and exophthalmos. Many of these patients initially present to orthopaedic surgeons, and misdiagnosis is frequent.
We deliver a case of a 10-month-old boy who consulted to our department. Previously misdiagnosed as a Kawasaki syndrome, TORCH, and osteomyelitis. He had undergone several examinations and had been discussed in clinocipathological conference (CPC) to narrow down the diagnosis.
After serial examinations, the diagnosis of Langerhans Cell Histiocytosis was confirmed and chemotherapy was initiated. And after 6 cycles of chemotherapy, with 1-week interval of each therapy, the clinical appearance of this patient significantly improved.
Despite major advances in our understanding and management of LCH, it remains one of the most challenging diagnoses for the orthopedic surgeon. By doing a comprehensive examination, it is possible to narrowing down the diagnosis and planning the accurate treatment.
朗格汉斯细胞组织细胞增多症（LCH）是一组病因尚不明确的罕见疾病。该疾病曾被称为组织细胞增多症X，临床表现广泛，包括嗜酸性肉芽肿（孤立性骨病变）、尿崩症和突眼症。许多此类患者最初就诊于骨科医生，误诊情况屡见不鲜。
我们报告一例10个月大男孩到我科就诊的病例。该患儿此前被误诊为川崎综合征、TORCH感染和骨髓炎。他接受了多项检查，并在临床病理讨论会上进行了讨论以明确诊断。
经过一系列检查，确诊为朗格汉斯细胞组织细胞增多症并开始化疗。经过6个周期的化疗，每个疗程间隔1周，该患者的临床表现显著改善。
尽管我们对LCH的认识和治疗取得了重大进展，但它仍然是骨科医生面临的最具挑战性的诊断之一。通过全面检查，有可能缩小诊断范围并制定准确的治疗方案。
