Özdemir Zeynep Maraş, Kahraman Ayşegül Sağır, Görmeli Cemile Ayşe, Sevimli Reşit, Akpolat Nusret
Department of Radiology, İnönü University School of Medicine, Malatya, Turkey.
Department of Orthopedics and Traumatology, İnönü University School of Medicine, Malatya, Turkey.
Balkan Med J. 2016 Sep;33(5):573-577. doi: 10.5152/balkanmedj.2016.160492. Epub 2016 Sep 1.
Langerhans cell histiocytosis (LCH), typically found in children, is a rare single or multisystem disorder with a wide range of clinical and radiological manifestations. Unusual presentations of LCH are occasionally encountered and it may be difficult to distinguish LCH from an infection or a benign or malignant tumor.
A 35-year-old female presented with pain in her back and left buttock, malaise, and weight loss, with a duration of several months. Her laboratory test results were within the normal ranges except for the levels of acute phase reactants, which were elevated. Magnetic resonance imaging and computed tomography revealed a unilateral destructive sacroiliac lesion, and multiple vertebral lesions with adjacent discal involvement and extensive soft tissue extensions. She was initially misdiagnosed with multifocal osteoarticular tuberculosis. An open biopsy and joint curettage was performed. Histopathological examination showed that she had LCH.
To the best of our knowledge, this is the first case of LCH associated with a destructive unilateral sacroiliac lesion, discal involvement, and involvement of the adjacent vertebrae, in an adult patient; the LCH mimicked osteoarticular tuberculosis. Disease onset in adulthood is rare, and this can potentially delay diagnosis. Familiarity with the imaging features of unusual LCH manifestations is necessary to ensure accurate diagnosis and appropriate treatment.
朗格汉斯细胞组织细胞增多症(LCH)通常见于儿童,是一种罕见的单系统或多系统疾病,具有广泛的临床和影像学表现。LCH的不寻常表现偶尔会遇到,可能难以将LCH与感染或良性或恶性肿瘤区分开来。
一名35岁女性出现背部和左臀部疼痛、不适及体重减轻,持续数月。除急性期反应物水平升高外,她的实验室检查结果均在正常范围内。磁共振成像和计算机断层扫描显示单侧骶髂关节破坏性病变,以及多个椎体病变,伴有相邻椎间盘受累和广泛的软组织延伸。她最初被误诊为多灶性骨关节结核。进行了开放活检和关节刮除术。组织病理学检查显示她患有LCH。
据我们所知,这是首例成年患者中与单侧骶髂关节破坏性病变、椎间盘受累及相邻椎体受累相关的LCH病例;该LCH酷似骨关节结核。成年期发病罕见,这可能会延迟诊断。熟悉不寻常LCH表现的影像学特征对于确保准确诊断和恰当治疗是必要的。