Acquired angioedema (AAE-C1INH) is a rare, potentially life-threatening disorder resulting from acquired C1 esterase inhibitor deficiency, often secondary to lymphoproliferative diseases such as splenic marginal zone lymphoma (SMZL). We report on a 68-year-old woman presenting with facial and tongue swelling, gastrointestinal symptoms, and splenomegaly. Laboratory work-up revealed low C1 esterase inhibitor and C1q complement levels, consistent with AAE-C1INH. Imaging showed marked splenomegaly with increased fluorodeoxyglucose uptake; bone marrow biopsy confirmed SMZL. Treatment with rituximab and bendamustine chemotherapy led to clinical improvement, normalization of complement levels, and cessation of angioedema episodes.
Dhulipalla et al. (Fri,) studied this question.