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孤立性胫骨病变作为朗格汉斯细胞组织细胞增生症的首发表现:两例报告并文献复习。

Solitary tibial lesion as the initial presentation of Langerhans cell histiocytosis: report of two cases and literature review.

机构信息

Department of Orthopaedics, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan.

Department of Orthopaedic Surgery, National Taiwan University Hospital, Taipei, Taiwan.

出版信息

J Int Med Res. 2021 Jan;49(1):300060520982826. doi: 10.1177/0300060520982826.

DOI:10.1177/0300060520982826
PMID:33472472
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7829610/
Abstract

The various presentations of osseous Langerhans cell histiocytosis (LCH) make it difficult to distinguish from other bone diseases. In addition, there is no universally accepted protocol for managing osseous LCH for single non-central nervous system-risk lesions. Here, the rare cases of two paediatric patients, aged 1 and 2 years, who presented with a solitary tibial lesion at time of LCH diagnosis, are reported. One patient progressed to multiple lesions after curettage of the original lesion. Subsequently, both patients received preventive chemotherapy using the Taiwan Paediatric Oncology Group (TPOG) revised protocol for treating low risk patients with LCH, namely, TPOG LCH2002-LR. After receiving this treatment, which included a schedule of prednisolone and vincristine for 6 weeks, followed by prednisolone, vincristine and 6-mercaptopurine for a further 48 weeks, both patients are free from recurrence or progression.

摘要

朗格汉斯细胞组织细胞增生症(LCH)的各种表现使其难以与其他骨骼疾病区分开来。此外,对于单个非中枢神经系统风险病变的骨骼 LCH,目前尚无普遍接受的管理方案。在此,报告了两例罕见的儿科患者,他们在 LCH 诊断时均表现为单一胫骨病变,年龄分别为 1 岁和 2 岁。一名患者在切除原始病变后进展为多个病变。随后,两名患者均根据台湾儿科肿瘤组(TPOG)治疗 LCH 低危患者的修订方案(即 TPOG LCH2002-LR)接受预防性化疗。接受包括泼尼松龙和长春新碱治疗 6 周,随后泼尼松龙、长春新碱和巯基嘌呤治疗 48 周的方案后,两名患者均未复发或进展。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f7f3/7829610/c55298ee8762/10.1177_0300060520982826-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f7f3/7829610/56cd23698a72/10.1177_0300060520982826-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f7f3/7829610/c55298ee8762/10.1177_0300060520982826-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f7f3/7829610/56cd23698a72/10.1177_0300060520982826-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f7f3/7829610/c55298ee8762/10.1177_0300060520982826-fig2.jpg

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