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抗合成酶综合征患者中未被怀疑的淋巴瘤样肉芽肿病。

Unsuspected lymphomatoid granulomatosis in a patient with antisynthetase syndrome.

作者信息

Skala Stephanie L, Hristov Alexandra C, Gudjonsson Johann E, Chan May P

机构信息

Department of Pathology, University of Michigan, Ann Arbor, USA.

Department of Dermatology, Department of Pathology, University of Michigan, Ann Arbor, USA.

出版信息

Cutis. 2017 Oct;100(4):E22-E26.

PMID:29136066
Abstract

Clinical diagnosis of lymphomatoid granulomatosis (LYG) often is difficult, especially in patients with multiple comorbidities. We present a 60-year-old woman with worsening fatigue, night sweats, unintentional weight loss, and dyspnea of 2 weeks' duration. Her medical history was remarkable for recent radiation therapy for recurrent breast cancer and antisynthetase syndrome complicated by interstitial lung disease and controlled with azathioprine. Computed tomography showed ground-glass opacities and nodular infiltrates in all lung lobes, raising concern for radiation pneumonitis and drug toxicity. Skin examination revealed erythematous and hemorrhagic papules, macules, and blisters on the lower leg. A diagnosis of LYG was made on a skin biopsy showing large angiocentric Epstein-Barr virus (EBV)-positive B cells. The patient soon progressed to develop nodal large B-cell lymphoma and died 6 weeks later. This rare report of LYG in a patient with antisynthetase syndrome highlights the diagnostic difficulty and aggressive course of LYG as well as the important role of skin biopsy in establishing the diagnosis.

摘要

淋巴瘤样肉芽肿病(LYG)的临床诊断通常很困难,尤其是在患有多种合并症的患者中。我们报告一名60岁女性,出现疲劳加重、盗汗、不明原因体重减轻和持续2周的呼吸困难。她的病史因近期复发性乳腺癌接受放射治疗以及抗合成酶综合征并发间质性肺病并使用硫唑嘌呤控制而引人注目。计算机断层扫描显示所有肺叶均有磨玻璃影和结节状浸润,这引起了对放射性肺炎和药物毒性的担忧。皮肤检查发现小腿有红斑、出血性丘疹、斑疹和水疱。皮肤活检显示大的血管中心性爱泼斯坦-巴尔病毒(EBV)阳性B细胞,从而确诊为LYG。该患者很快进展为结节性大B细胞淋巴瘤,并在6周后死亡。这例抗合成酶综合征患者中LYG的罕见报告突出了LYG的诊断困难和侵袭性病程以及皮肤活检在确立诊断中的重要作用。

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