Acquired angioedema in B cell lymphoproliferative disease: A retrospective case series.
Clin Exp Immunol
; 206(3): 378-383, 2021 12.
Article
in En
| MEDLINE
| ID: mdl-34586637
ABSTRACT
Acquired angioedema due to C1-inhibitor (C1-INH) deficiency (AAE-C1-INH) is rare and is associated with underlying lymphoproliferative diseases. C1-INH deficiency may be due to neoplastic over-consumption of C1-INH and the generation of anti-C1-INH autoantibodies. Uncovering an occult malignancy can lead to earlier oncology referral and improvement of angioedema after treatment of the underlying lymphoproliferative disorder. We characterized seven patients with C1-INH-AAE that highlights the importance of recognizing the association between C1-INH-AAE and underlying malignancy. In acute attacks, patients may be resistant to C1-INH therapy due to the presence of anti-C1-INH autoantibodies or rapid complement consumption, and may respond better to icatibant or ecallantide, which directly affect bradykinin. Treatment of the underlying malignancy also improves AAE-C1-INH symptoms and supports the role of lymphoproliferative B cells in AAE-C1-INH pathophysiology. Monitoring levels of C4, C1-INH function and level, and C1q may be predictive of AAE-C1-INH control and be used as surrogates for treatment efficacy. With close monitoring, low-dose danazol can be effective for long-term prophylaxis. Annual evaluation in AAE-C1-INH is recommended if an underlying malignancy is not found, as angioedema may precede the development of malignancy by several years. Our single-center study has aided in standardization of comprehensive AAE-C1-INH diagnosis, treatment, and monitoring strategies towards future therapeutic clinical trials.
Key words
Full text:
1
Database:
MEDLINE
Main subject:
Complement C1 Inhibitor Protein
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Hereditary Angioedema Types I and II
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Angioedema
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Lymphoproliferative Disorders
Type of study:
Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limits:
Aged
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Female
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Humans
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Male
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Middle aged
Language:
En
Journal:
Clin Exp Immunol
Year:
2021
Type:
Article
Affiliation country:
United States