CAJ | 학술논문

目的：报道1例罕见的儿童皮肌炎伴发骨髓异常增生综合征的诊断、鉴别诊断和治疗经过。方法分析患儿的发病经过、临床特点和诊疗过程，对患儿的骨髓、淋巴结和肌肉标本进行包括病理学，流式细胞术以及免疫组织化学在内的系列检测。结果根据患儿临床表现和相关实验室检查结果可诊断为儿童皮肌炎，依据患儿伴随的血液学改变，诊断为骨髓异常增生综合征。结论伴发骨髓异常增生综合征的儿童皮肌炎非常特殊和罕见，其发病机制和诊疗策略尚待进一步探索。
목적：보도1례한견적인동피기염반발골수이상증생종합정적진단、감별진단화치료경과。방법분석환인적발병경과、림상특점화진료과정，대환인적골수、림파결화기육표본진행포괄병이학，류식세포술이급면역조직화학재내적계렬검측。결과근거환인림상표현화상관실험실검사결과가진단위인동피기염，의거환인반수적혈액학개변，진단위골수이상증생종합정。결론반발골수이상증생종합정적인동피기염비상특수화한견，기발병궤제화진료책략상대진일보탐색。
Objectives A rare case of juvenile dermatomyositis (JDM) complicated by myelodysplastic syndrome (MDS) was reported in order to improve the understanding of the disease in its clinical signs, differential diagnosis and treatment. Meth-ods The course of the disease, clinical characteristics and the process of diagnosis and treatment were analyzed. Specimens of bone marrow, lymph nodes, muscles were examined by histopathological or immunohistopathological means and lfow cytometry. Results The diagnosis of JDM complicated by MDS was made based on the clinical manifestations, related laboratory results and the accompanying changes in hematological and bone marrow examinations. Conclusions JDM complicated by MDS is very special and rarely seen in China and its pathological mechanism and strategy of diagnosis and treatment should be further explored.