Acquired C1-inhibitor deficiency presenting with nephrotic syndrome. Issue 7 (11th July 2019)

• Record Type: Journal Article
• Authors: Willows, Jamie; Wood, Katrina; Bourne, Helen; Sayer, John Andrew
• Is Part Of: BMJ case reports. Volume 12:Issue 7(2019)
• Publication Date: 2019-07-11
• Subjects: renal system -- immunology -- haematology (incl blood transfusion); Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2019-230388
• Languages: English
• ISSNs: 1757-790X

Abstract

Abstract : 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.