Willows Jamie, Wood Katrina, Bourne Helen, Sayer John Andrew
Renal Services, Freeman Hospital, Newcastle upon Tyne, UK.
Histopathology Department, Royal Victoria Infirmary, Newcastle upon Tyne, UK.
BMJ Case Rep. 2019 Jul 11;12(7):e230388. doi: 10.1136/bcr-2019-230388.
Acquired C1-inhibitor (C1-INH) deficiency is a rare and potentially life-threatening disorder, which presents with recurrent attacks of non-pitting oedema to the face, airway, limbs or gastrointestinal tract. It is often associated with underlying B-cell lymphoproliferative disorders. We describe a case of a 73-year-old man with acquired C1-INH deficiency who presented with nephrotic syndrome due to glomerular IgM deposition, secondary to an underlying secretory lymphoplasmacytic lymphoma. Both the acquired C1-INH deficiency and the nephrotic syndrome resolved when the underlying B-cell lymphoma was treated with rituximab and bendamustine, suggesting the underlying lymphoproliferative malignancy was driving both disorders.
获得性C1抑制物（C1-INH）缺乏症是一种罕见且可能危及生命的疾病，表现为面部、气道、四肢或胃肠道反复出现非凹陷性水肿。它常与潜在的B细胞淋巴增殖性疾病相关。我们描述了一例73岁男性获得性C1-INH缺乏症患者，该患者因潜在的分泌性淋巴浆细胞淋巴瘤继发肾小球IgM沉积而出现肾病综合征。当用利妥昔单抗和苯达莫司汀治疗潜在的B细胞淋巴瘤时，获得性C1-INH缺乏症和肾病综合征均得到缓解，这表明潜在的淋巴增殖性恶性肿瘤是导致这两种疾病的原因。
