儿童皮肌炎伴发骨髓异常增生综合征1例报告

摘要

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.%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。