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Acquired angioedema: an unusual presentation of haematological malignancy
  1. Daniel Farrugia,
  2. Michael Caruana Dingli and
  3. Mark Grech
  1. Haematology, Mater Dei Hospital, Msida, Malta
  1. Correspondence to Dr Michael Caruana Dingli; michael.caruana-dingli{at}


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.

  • Haematology (incl blood transfusion)
  • Immunology
  • Haematology (drugs and medicines)

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  • Contributors DF: write up, literature review and editing of images. MCD: write up, literature review and editing of images. MG: review of write up with amendments as necessary and editing of images.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.