D2.43 - Hereditary Angioedema Can Hit at Any Age: A Case Report in Egypt

Background Hereditary angioedema (HAE) is a rare, potentially life-threatening condition characterised by swelling of the face, tongue, throat, and extremities due to increased levels of the bradykinin peptide. Unlike allergic reactions, it is typically non-itchy and lacks hives.

Case Presentation A 76-year-old male patient presented with history of recurrent facial oedema of 6 months duration, that was persistent more than 72hr without hives and poor response to antihistamines. Past history was negative for laryngeal attacks or abdominal pain, but positive for hypertension (Valsartan), diabetes mellitus (Vildagliptin), and autoimmune haemolytic anaemia with use of systemic steroids for a year, but the patient was lost to follow-up. Negative family history of HAE. Laboratory findings were significant for Low serum levels of C4, C1 esterase inhibitor level and activity, and high serum levels of immunoglobulin M and free light chains with serum immunofixation revealing clonality at Kappa light chain regions, with positive anti-nuclear antibody (Titer of 1/80, nuclear dense fine speckled pattern) and Coombs test, but negative Anti-C1Q & Anti-double-stranded DNA antibodies and normal levels of C1Q. Bone marrow biopsy revealed infiltration with lymphoid population showing pan B positivity and low proliferation index, in keeping with stage-IV B-Non-Hodgkin lymphoma. HAE-Type 1 diagnosis was made and the patient was kept on long-term prophylaxis with Lanadelumab 300mg subcutaneous every 2 weeks for 6 months and intravenous plasma-derived C1- Concentrate as on-demand protocol.

Conclusion HAE attacks can manifest at any age, though symptoms most commonly begin in childhood or adolescence. Angioedema could be the iceberg of haematological malignancy as early presentation, especially in old age.