年轻女性朗格汉斯细胞组织细胞增多症伴多系统累及:1例报告及文献复习

Sheniz Yuzeir, Hinko Varbanov, T. Yordanova, I. Micheva
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摘要

朗格汉斯细胞组织细胞增生症(LCH)又称X型组织细胞增生症(HX),是一组病因不明的增生性细胞疾病。LCH会影响骨骼、肺部、中枢神经系统、肝脏、胸腺、皮肤和淋巴结。LCH的诊断很难执行,在成年人中很少发现,每年每百万人中只有大约5例。本研究报告一例年轻女性LCH多系统受累,包括骨、眼眶、肺系统和中枢神经系统。患者接受化疗6个月,受累系统改善迅速。最后一次PET/CT显示右髂骨代谢活动。治疗结束一年后,患者返回医院,显示健康状况恶化。这个临床病例很有趣,不仅因为组织细胞增多症的临床、形态学和影像学资料,还因为这种现象的预后和诊断重要性尚不明确。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Langerhans Cell Histiocytosis with Multisystem Involvement in a Young Woman: A Case Report and Literature Review
Langerhans Cell Histiocytosis (LCH), also known as Histiocytosis X (HX), is a group of hyperplastic cellular diseases of unknown causes. LCH could affect bones, lungs, central nervous system, liver, thymus, skin, and also lymph nodes. The diagnosis of LCH is difficult to enforce and rarely found in adults, with just about 5 cases per million per year. The present study reports the case of a young woman with LCH with multisystem involvement, including that of the bone, orbit, pulmonary system and central nervous system. The patient received chemotherapy for 6 months and exhibited rapid improvement in the involved systems. The last PET/CT showed metabolic activity in the right iliac bone. One year after completion of the therapy, the patient returned to the hospital showing deteriorating health. The clinical case is interesting not only because of the registered clinical, morphological, and imaging data of histiocytosis but also because of the unclear prognostic and diagnostic importance of this phenomenon.
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