Nugraha Harry Galuh, Muslimah Astri Restuastuti, Suryawan Nur, Usman Hermin Aminah
Department of Radiology, Faculty of Medicine, Universitas Padjadjaran, Dr. Hasan Sadikin General Hospital, Bandung, Indonesia.
Department of Pediatric, Faculty of Medicine, Universitas Padjadjaran, Dr. Hasan Sadikin General Hospital, Bandung, Indonesia.
Radiol Case Rep. 2024 Sep 27;19(12):6520-6527. doi: 10.1016/j.radcr.2024.09.021. eCollection 2024 Dec.
Langerhans cell histiocytosis (LCH) is a rare clonal disorder of the monocyte-macrophage system. There are 3 distinct types of LCH: multisystem type (MS), single-system multi-site (SS-m), and single-system single-site (SS-s). The disease is most common in young children, especially males. In this case, we report the rare case of a young Asian girl who initially complained of dyspnea, diarrhea, rash, and fever. Upon further examination, multiple systems were implicated, including the bone, skin, lung, liver, and spleen. Multisystem LCH with risk organ involvement give poor prognosis to the patients.
朗格汉斯细胞组织细胞增多症(LCH)是一种罕见的单核细胞 - 巨噬细胞系统克隆性疾病。LCH有3种不同类型:多系统型(MS)、单系统多部位型(SS - m)和单系统单部位型(SS - s)。该疾病在幼儿中最为常见,尤其是男性。在此病例中,我们报告了一名年轻亚洲女孩的罕见病例,她最初主诉呼吸困难、腹泻、皮疹和发热。进一步检查发现多个系统受累,包括骨骼、皮肤、肺、肝脏和脾脏。伴有风险器官受累的多系统LCH患者预后较差。