本文選題：肥大細(xì)胞增生癥 + 播散性肥大細(xì)胞增生癥　； 參考：《山東醫(yī)藥》2017年39期

【摘要】：目的分析總結(jié)以大皰為主要表現(xiàn)的肥大細(xì)胞增生癥的診斷、治療方法和預(yù)后情況。方法回顧性分析12例以大皰為主要表現(xiàn)的肥大細(xì)胞增生癥患兒的臨床資料,包括臨床癥狀、體征、相關(guān)檢驗(yàn)項(xiàng)目結(jié)果(血液分析、胸部X線片檢查、腹部彩超檢查、皮膚活組織病理檢查及骨髓穿刺活檢),治療及預(yù)后資料。結(jié)果 12例患兒臨床表現(xiàn)相似,Darier征均陽(yáng)性。除大皰外,還可有皮膚橘皮樣改變,面部潮紅,其中3例有暈厥史。胸部X線片、腹部彩超、長(zhǎng)骨拍片檢查和骨髓穿刺活檢等系統(tǒng)性檢查均未見明顯損害。根據(jù)臨床表現(xiàn)和病理檢查發(fā)現(xiàn)真皮有肥大細(xì)胞浸潤(rùn)、肥大細(xì)胞有異染顆粒而確診。根據(jù)患兒臨床癥狀予以抗過(guò)敏、激素治療及對(duì)癥支持治療,8例在1歲半到3歲半停藥后痊愈、未復(fù)發(fā),3例用藥至3歲半病情仍有反復(fù),1例患兒失訪。結(jié)論以大皰為主要表現(xiàn)的肥大細(xì)胞增生癥比較罕見,具有特征的臨床表現(xiàn),結(jié)合病理檢查可確診。治療以抗過(guò)敏藥物和對(duì)癥支持治療為主,預(yù)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.
【作者單位】： 華中科技大學(xué)同濟(jì)醫(yī)學(xué)院附屬武漢兒童醫(yī)院武漢市婦幼保健院;
【分類號(hào)】：R725.5

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