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一名儿童的多灶性朗格汉斯细胞组织细胞增生症

Multifocal Langerhans cell histiocytosis in a child.

作者信息

Musa Juna, Siddik Abu Bakar, Ahmetgjekaj Ilir, Rahman Masum, Guy Ali, Rahman Abdur, Ikram Samar, Kola Erisa, Vokshi Valon, Ahsan Eram, Saliaj Kristi, Hyseni Fjolla

机构信息

Department of General Surgery, Mayo Clinic, Rochester, MN, USA.

Department of Pain Medicine, Mayo Clinic, Jacksonville, Florida, USA.

出版信息

Radiol Case Rep. 2021 Jul 17;16(9):2726-2730. doi: 10.1016/j.radcr.2021.06.052. eCollection 2021 Sep.

Abstract

Langerhans Cell Histiocytosis (LCH) is a rare disorder sometimes called the disorder of the "monocyte-macrophage system". This condition is characterized by the proliferation of abnormal Langerhans cells within different tissues. Skin rash is the typical early feature, but bony involvement is the second most common presentation. The most common complications are musculoskeletal disabilities, hearing problems, skin scarring, neuropsychiatric defects and most importantly, progression to secondary malignancies like leukemia. Early recognition and treatment can reduce morbidity and mortality. Herein, we report a case of a 10-year-old male presenting with a tender, palpable mass in the lower limb. On initial imaging, a lesion involving the diaphysis of the fibula was observed, raising concerns of Ewing sarcoma. Biopsy was planned along with whole-body MRI, revealing multifocal single system Langerhans cell histiocytosis. Given the rarity of fibular involvement in LCH, distinguishing between LCH and common malignancies within this age-group can be challenging. Through this case report, we hope to emphasize the importance of considering LCH in the differential diagnosis to ensure a timely diagnosis, fitting treatment and improvement in prognosis of the condition.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种罕见疾病,有时被称为“单核巨噬细胞系统疾病”。这种病症的特征是不同组织内异常朗格汉斯细胞的增殖。皮疹是典型的早期特征,但骨骼受累是第二常见的表现。最常见的并发症是肌肉骨骼残疾、听力问题、皮肤瘢痕形成、神经精神缺陷,最重要的是进展为继发性恶性肿瘤,如白血病。早期识别和治疗可降低发病率和死亡率。在此,我们报告一例10岁男性病例,其下肢出现压痛性可触及肿块。初步影像学检查发现腓骨干骺端有病变,引起了尤因肉瘤的担忧。计划进行活检并进行全身MRI检查,结果显示为多灶性单系统朗格汉斯细胞组织细胞增多症。鉴于LCH累及腓骨的情况罕见,在该年龄组中区分LCH与常见恶性肿瘤可能具有挑战性。通过本病例报告,我们希望强调在鉴别诊断中考虑LCH的重要性,以确保及时诊断、恰当治疗并改善病情预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e489/8318828/2e1a1f6a8eba/gr1.jpg

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