Acquired angioedema: an unusual presentation of haematological malignancy

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.

Keywords: Haematology (drugs and medicines); Haematology (incl blood transfusion); Immunology.

Figure 1

Composite image where images (A) and (B), respectively, represent ×100 and ×400 magnification images of the splenic biopsy showing congestion. Images (C) and (D), respectively, represent ×100 and ×400 magnification images of bone marrow showing a subtle lymphocytic intrasinusoidal infiltrate in a single filing pattern on CD20 staining.

Figure 2

Increased diffuse splenic tracer uptake and splenomegaly on positron emission tomography/CT scan.