Acquired Angioedema and Chronic Lymphocytic Leukemia: Unraveling the Complex Interplay and Addressing Refractory Cases
Acquired angioedema (AAE) due to deficiency of a C1 esterase inhibitor (C1-INH; AAE-C1-INH) is a rare and potentially fatal syndrome characterized by recurrent episodes of angioedema without urticaria. Often underdiagnosed due to its rarity and mimicry of common allergic reactions, AAE-C1-INH is ass...
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| Published in: | Cureus Vol. 15; no. 12; p. e50238 |
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| Main Authors: | , |
| Format: | Report |
| Language: | English |
| Published: |
01-12-2023
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| Online Access: | Get full text |
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| Summary: | Acquired angioedema (AAE) due to deficiency of a C1 esterase inhibitor (C1-INH; AAE-C1-INH) is a rare and potentially fatal syndrome characterized by recurrent episodes of angioedema without urticaria. Often underdiagnosed due to its rarity and mimicry of common allergic reactions, AAE-C1-INH is associated with lymphoproliferative disorders, necessitating early recognition for improved outcomes. We present a case of a 63-year-old male diagnosed with AAE-C1-INH and concurrent stage 0 chronic lymphocytic leukemia (CLL), a rarely documented association. Despite chemotherapy, the patient experienced persistent angioedema until C1 esterase inhibitor therapy was initiated. This case underscores the importance of screening for lymphoproliferative disorders in AAE-C1-INH patients and explores refractory cases, urging further research into mechanisms and treatment strategies. |
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| Bibliography: | ObjectType-Case Study-2 content type line 59 SourceType-Reports-1 ObjectType-Report-1 |
| ISSN: | 2168-8184 2168-8184 |
| DOI: | 10.7759/cureus.50238 |