Angioedema in lymphoproliferative disease: A case report

Abstract A link between adult onset recurrent angioedema and lymphoproliferative disease has been noted previously. The underlying mechanism(s) may be explained by involvement of the contact and complement system, or autoimmunity, or a combination thereof. We report the case of a 64-year-old patient who experienced symptoms of angioedema following a recent diagnosis of diffuse large B-cell lymphoma. A total of two such episodes were encountered in this patient, with rapid response to steroids and supportive care. In the context of acute, aggressive lymphoma, angioedema was retrospectively concerning for acquired C1 inhibitor deficiency. Our patient’s older age, lack of a personal and family history of similar episodes, lack of an allergic trigger and of pruritis and hives, proximity in time to lymphoma diagnosis, as well as length of angioedema symptoms between 2–5 days supported this diagnosis. The window for laboratory confirmation of this entity is narrow, underscoring the importance of clinical awareness and timely recognition.