Acquired angioedema: an unusual presentation of haematological malignancy. Issue 9 (19th September 2022)

• Record Type: Journal Article
• Authors: Farrugia, Daniel; Caruana Dingli, Michael; Grech, Mark
• Is Part Of: BMJ case reports. Volume 15:Issue 9(2022)
• Publication Date: 2022-09-19
• Subjects: Haematology (incl blood transfusion) -- Immunology -- Haematology (drugs and medicines); Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2022-249093
• Languages: English
• ISSNs: 1757-790X

Abstract

Abstract : A previously healthy man in his 60s being worked up for splenomegaly presented to the emergency department with recurrent episodes of angioedema. Each episode was attributed to a precipitating cause, and consequently, the predisposing C1 esterase inhibitor (C1-INH) deficiency remained undiagnosed until the third presentation. The aetiology of acquired C1-INH deficiency would be primarily obscure and require further investigations to identify. A clonal B cell population was finally isolated by flow cytometry after multiple repeat marrow samples, and a diagnosis of splenic marginal zone lymphoma was subsequently reached. Response to single-agent rituximab was observed with resolution of splenomegaly, disappearance of the antibody and restoration of C1-INH levels.