CAJ | 학술논문

目的：探讨多系统朗格汉斯细胞组织细胞增生症(multi-system Langerhans cell system，MS-LCH)的临床特点、病理诊断、鉴别诊断及预后等。方法：结合相关文献，对8例MS-LCH的临床资料、病理学表现和免疫组织化学结果进行分析。结果：男性1例，女性7例，年龄从13 d~3岁5个月，其中6例累及高危器官。临床表现为发热、皮疹或贫血，伴有肝、脾、淋巴结、骨、垂体等多系统器官受累。镜下见受累器官内朗格汉斯组织细胞增生，核呈分叶状或不规则形。免疫组化显示朗格汉斯细胞阳性表达CD1a和S-100。结论：MS-LCH临床表现复杂，诊断需要结合临床表现、影响学检查及组织病理学检查。累及高危器官者，预后较差。
목적：탐토다계통랑격한사세포조직세포증생증(multi-system Langerhans cell system，MS-LCH)적림상특점、병리진단、감별진단급예후등。방법：결합상관문헌，대8례MS-LCH적림상자료、병이학표현화면역조직화학결과진행분석。결과：남성1례，녀성7례，년령종13 d~3세5개월，기중6례루급고위기관。림상표현위발열、피진혹빈혈，반유간、비、림파결、골、수체등다계통기관수루。경하견수루기관내랑격한사조직세포증생，핵정분협상혹불규칙형。면역조화현시랑격한사세포양성표체CD1a화S-100。결론：MS-LCH림상표현복잡，진단수요결합림상표현、영향학검사급조직병이학검사。루급고위기관자，예후교차。
Objective:To study clinical and histopathological characteristics, diagnostic criteria, differential diagnosis and prognosis of systematic Langerhans cell histiocytosis in children.Methods: Clinical and pathological features were studied in 8 cases of multi-system Langerhans cell histiocytosis with review of the literatures.Results:hTere were 1 males and 7 females. hTe youngest was 13-day-old and the oldest was 3 years and 5 months old. Among those MS-LCH patients, 6 patients had risk organ involvement. The clinical symptoms included fever, skin eruption or anemia, with inifltrative lesion of liver, spleen, lymph node, bone and hematopoietic system. Microscopically, the disease was characterized by clonal hyperplasia of histiocytes with folded nucleus. Immunohistochemical staining showed that the histiocytes were positive for CD1a and S-100.Conclusion: Multi-system Langerhans cell histiocytosis presents complex clinical symptoms. hTe diagnosis needs a combination of clinical features, image examination and histopathological characters. If the risk organ was involved, the prognosis is poor.