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一名患有骨髓增生异常综合征、难治性贫血且原始细胞增多的老年患者发生大疱性类天疱疮。

Bullous pemphigoid in an elderly patient with myelodysplastic syndrome and refractory anemia coupled with excess of blast.

作者信息

Lee Yin Yin, Bee Ping Chong, Lee Chew Kek, Naiker Manimalar, Ismail Rokiah

机构信息

Department of Medicine, University of Malaya, Kuala Lumpur, Malaysia.

出版信息

Ann Dermatol. 2011 Dec;23(Suppl 3):S390-2. doi: 10.5021/ad.2011.23.S3.S390. Epub 2011 Dec 27.

Abstract

Bullous pemphigoid (BP) has a recognized association with solid organ tumors, but is relatively rare in hematological malignancies. We report a 67-year-old male who developed BP after being diagnosed with myelodysplastic syndrome and refractory anemia with excess of blast (RAEB). Skin biopsy elucidated sub-epidermal bulla using direct immunofluorescence, revealing linear C3 and IgG deposits along the basement membrane. His BP was recalcitrant to the conventional treatment and only responded to a combination of high dose oral prednisolone and azathioprine. The relative refractory nature of his condition and concurrent RAEB supports a paraneoplastic nature.

摘要

大疱性类天疱疮(BP)与实体器官肿瘤存在公认的关联,但在血液系统恶性肿瘤中相对少见。我们报告一名67岁男性,在被诊断为骨髓增生异常综合征及伴原始细胞增多的难治性贫血(RAEB)后发生了BP。皮肤活检通过直接免疫荧光显示表皮下大疱,揭示沿基底膜有线性C3和IgG沉积。他的BP对传统治疗耐药,仅对高剂量口服泼尼松龙和硫唑嘌呤联合治疗有反应。其病情的相对难治性及并发的RAEB支持副肿瘤性本质。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/50bf/3276805/05a587d1aeef/ad-23-S390-g001.jpg

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