Acquired Angioedema Can Be First Symptom of Underlying Conditions Such As Lymphoma, Case Reports Suggests

Acquired angioedema can be the first symptom of underlying conditions such as lymphoma. In those cases, cancer treatment may be the best approach to stop angioedema manifestations, a case report suggests.

The study, “A Case of Acquired Angioedema with Low C1 Inhibitor (C1-INH) Associated with Splenic Marginal Zone Lymphoma,” was published in the American Journal of Case Reports.

Acquired angioedema usually appears in people older than 40 who have low levels of C1 esterase inhibitor (C1-INH). This protein regulates bradykinin, a substance that causes swelling. Autoimmune diseases and blood conditions, such as non-Hodgkin’s lymphoma, tend to be the underlying cause of the reduction of C1-INH levels.

Researchers in Canada described the case of a 68-year-old man who developed acquired angioedema caused by non-Hodgkin’s lymphoma.

The man went to the emergency room with swelling in his tongue and lips. He had no redness or hives on the skin, itchy sensation, difficulty breathing, or abdominal pain. The swelling resolved over the next day without treatment.

The man reported having depression, high blood pressure, and low platelet count for the past one-and-a-half years. He had been treated for testicular cancer 30 years before.

Lab exams showed low levels of platelets, red, and white blood cells, without any apparent cause. Further exams, including a bone marrow biopsy, showed that the man had splenic marginal zone lymphoma, a type of non-Hodgkin’s lymphoma.

A careful examination of the man’s past medical history revealed he had experienced other episodes of angioedema, such as swelling in the lower limbs and abdominal pain. All the events resolved without treatment after a few days. The man’s lab exams showed low C1-INH levels, which confirmed the diagnosis of acquired angioedema.

The patient started therapy with danazol to prevent further angioedema attacks. Over the following months, he had mild fatigue and reflux. The man also had an enlarged spleen, which doubled in size and started causing discomfort. Blood cell levels also worsened.

The patient underwent surgery to remove his spleen one-and-a-half years after the initial diagnosis. The surgery aimed to improve blood cell count and to treat the lymphoma. The man stopped taking danazol shortly after the surgery.

The levels of blood cells improved soon after the surgery. The man did not present any angioedema attacks and remained in remission without any further treatment at least eight months after the surgery.

The researchers concluded that “in this case, [acquired] angioedema with recurrent episodes of soft tissue swelling was associated with underlying hematologic malignancy [lymphoma]. The patient’s angioedema resolved when the malignancy was treated.”

They also noted that in cases of angioedema associated with lymphoma the best approach to reduce angioedema attacks is to induce lymphoma remission.