以大疱为主要表现的儿童肥大细胞增生症12例分析

发布时间：2018-06-21 06:42

本文选题：肥大细胞增生症 + 播散性肥大细胞增生症　；参考：《山东医药》2017年39期

【摘要】：目的分析总结以大疱为主要表现的肥大细胞增生症的诊断、治疗方法和预后情况。方法回顾性分析12例以大疱为主要表现的肥大细胞增生症患儿的临床资料,包括临床症状、体征、相关检验项目结果(血液分析、胸部X线片检查、腹部彩超检查、皮肤活组织病理检查及骨髓穿刺活检),治疗及预后资料。结果 12例患儿临床表现相似,Darier征均阳性。除大疱外,还可有皮肤橘皮样改变,面部潮红,其中3例有晕厥史。胸部X线片、腹部彩超、长骨拍片检查和骨髓穿刺活检等系统性检查均未见明显损害。根据临床表现和病理检查发现真皮有肥大细胞浸润、肥大细胞有异染颗粒而确诊。根据患儿临床症状予以抗过敏、激素治疗及对症支持治疗,8例在1岁半到3岁半停药后痊愈、未复发,3例用药至3岁半病情仍有反复,1例患儿失访。结论以大疱为主要表现的肥大细胞增生症比较罕见,具有特征的临床表现,结合病理检查可确诊。治疗以抗过敏药物和对症支持治疗为主,预后良好。
[Abstract]:Objective to analyze and summarize the diagnosis, treatment and prognosis of mast cell hyperplasia with bullae as the main manifestation. Methods the clinical data of 12 cases of mast cell hyperplasia with bullae were analyzed retrospectively, including clinical symptoms, signs and related results (blood analysis, chest X-ray examination, abdominal color Doppler examination). Skin biopsy and bone marrow biopsy, treatment and prognosis. Results the clinical manifestations of 12 cases were similar to that of Darier sign. In addition to bullous, there can be skin orange peel changes, facial flashes, including 3 cases of syncope history. No obvious damage was found in chest X-ray, abdominal color Doppler, long bone examination and bone marrow biopsy. According to clinical manifestation and pathological examination, mast cell infiltration and metachromatic granules were found in dermis. According to the clinical symptoms of the children, 8 cases were cured by hormone therapy and symptomatic support therapy after withdrawal of drugs from 1 year old to 3 and a half years old, 3 cases without recurrence and 3 and a half years old. Conclusion Mast cell hyperplasia with bullae as the main manifestation is rare and has the characteristic clinical manifestation and can be diagnosed with pathological examination. The main treatment was antiallergic drugs and symptomatic support therapy, and the prognosis was good.
【作者单位】：华中科技大学同济医学院附属武汉儿童医院武汉市妇幼保健院;
【分类号】：R725.5

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