Bidkar Vijay G, Rajan Nita Rachel, Dasar Santosh, Naik Ashok S, Rao Ravikala
Department of Otorhinolaryngology Head and Neck Surgery, Shri Dharmasthala Manjunatheshwara College of Medical Sciences and Hospital, Sattur, Dharwad, Karnataka 580009 India.
Department of Radiology, Shri Dharmasthala Manjunatheshwara College of Medical Sciences and Hospital, Sattur, Dharwad, Karnataka India.
Indian J Otolaryngol Head Neck Surg. 2019 Oct;71(Suppl 1):96-99. doi: 10.1007/s12070-017-1122-5. Epub 2017 Apr 7.
The clinical presentation except age of onset is similar in different types of angioedema. A lymphoproliferative disorder like angioimmunoblastic T cell lymphoma (AITL) rarely presents with symptoms of angioedema. We present extremely rare case of elderly male with recurrent tongue swelling, pruritus with normal levels of complements and C1 esterase inhibitor protein featuring as acquired angioedema, a rare manifestation of AITL. Initial response to corticosteroids may be misleading and occurs as a result of immunosuppression of AITL. High index of suspicion may prompt need for histopathological diagnosis of lymph node biopsy. Definitive chemotherapeutic treatment may achieve long term remission.
除发病年龄外，不同类型血管性水肿的临床表现相似。像血管免疫母细胞性T细胞淋巴瘤（AITL）这样的淋巴增殖性疾病很少表现出血管性水肿的症状。我们报告了一例极为罕见的老年男性病例，该患者反复出现舌部肿胀、瘙痒，补体和C1酯酶抑制蛋白水平正常，表现为获得性血管性水肿，这是AITL的一种罕见表现。对皮质类固醇的初始反应可能具有误导性，是AITL免疫抑制的结果。高度怀疑可能促使需要对淋巴结活检进行组织病理学诊断。确定性的化疗可能实现长期缓解。
