[Acquired angioedema associated with non-Hodgkin lymphoma: a clinical case report].

Abstract

Background: Acquired angioedema (AEA) is rare and usually appears after the fourth decade of life. It is characterized by recurrent episodes of nonpuriginous angioedema affecting the skin, gastrointestinal tract, and upper respiratory tract, making it difficult to distinguish from hereditary angioedema.

Case report: Clinical presentation: A 44-year-old man with a history of non-Hodgkin lymphoma (NHL) in 2021, which was remitted. He presented with facial angioedema lasting 5 hours in June 2024, painless and non-puriginous. He denies recognizing any environmental factors, medications, or foods that exacerbate the condition, nor does he have a history of atopy. Laboratory/imaging studies: normal bile duct, IgE, ANA, C3, and C4. Thyroid profile was unremarkable. ESR and stool samples were normal. Autologous serum albumin (ASL) was negative. Physical urticaria tests were negative. C1 inhibitor was pending. Possible infectious processes were ruled out. Outcome: Partial improvement with antihistamines. In January 2025, the patient presented with a dry cough, which progressed to hemoptysis. A chest CT scan demonstrated a tumor in the right apex. Bronchoscopy with biopsy confirmed infiltration by NHL.

Conclusion: There are two forms of AEA: type I with a recognized association with lymphoproliferative disorders, with NHL accounting for 20% of cases. It can precede the onset of NHL by an average of 2.3 years; in this case, it was the first manifestation of relapse. She has currently started chemotherapy without further episodes of angioedema. Late-onset angioedema in adulthood can be associated with a wide variety of underlying diseases, with hematologic malignancies being a significant group. A deliberate search for clinical data on malignancies leads to their timely identification.