A rare case of high risk multisystem Langerhans Cell Histiocytosis in infant

Seerwan Hamadameen Sulaiman, Halit Demir
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Abstract

Objective: Langerhans cell histiocytosis (LCH) stands as the most common histiocytic disorder. It is categorized into single-system single site, single-system multi-site, and multisystem types, with or without involvement of risk organs. Clinical presentation exhibits considerable variability, contingent upon the affected organ systems. Given its rarity, diverse locations, and varying severity, there are currently no established treatment guidelines for LCH. Case Presentation: We documented the case of a 5-month-old male diagnosed with Langerhans cell histiocytosis (LCH), presenting with high-risk multisystem organ involvement and concurrent unifocal bone involvement. Bone marrow aspiration revealed infiltration by multinucleated giant cells within the marrow. Skin biopsy demonstrated Langerhans cell proliferation with epidermotrophism and the characteristic reniform nucleus resembling a coffee bean. The patient was treated following the histiocytosis protocol, which included daily oral prednisone and 6-mercaptopurine, in addition to weekly intravenous vinblastine and methotrexate administered every two weeks. Unfortunately, the patient displayed limited response during the initial phase of treatment and, tragically, passed away during the eighth week of therapy. Conclusion: Langerhans cell histiocytosis (LCH) is a rare disease characterized by variable disease presentations and is associated with significant morbidity and mortality. Timely recognition of clinical manifestations and risk factors, coupled with appropriate treatment, is crucial to reduce mortality and long-term complications associated with the condition.
罕见的婴儿多系统朗格汉斯细胞组织细胞增多症1例
目的:朗格汉斯细胞组织细胞增多症(LCH)是最常见的组织细胞疾病。分为单系统单部位型、单系统多部位型和多系统型,有或无危险器官参与。临床表现具有相当大的可变性,取决于受影响的器官系统。鉴于其罕见性、不同的位置和不同的严重程度,目前尚无针对LCH的既定治疗指南。病例介绍:我们记录了一个5个月大的男性诊断为朗格汉斯细胞组织细胞增多症(LCH)的病例,表现为高风险的多系统器官受累和并发的单发骨受累。骨髓穿刺示骨髓内多核巨细胞浸润。皮肤活检显示朗格汉斯细胞增生,具有表皮滋养性和典型的肾形细胞核,类似于咖啡豆。患者接受组织细胞增生治疗方案,包括每日口服强的松和6-巯基嘌呤,以及每周静脉注射vinblastine和甲氨蝶呤,每两周给药一次。不幸的是,患者在治疗初期表现出有限的反应,不幸的是,在治疗的第八周去世了。结论:朗格汉斯细胞组织细胞增多症(LCH)是一种罕见的疾病,其特点是疾病表现多样,发病率和死亡率高。及时识别临床表现和危险因素,加上适当的治疗,对于降低死亡率和与该病相关的长期并发症至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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