Angioedema with normal C1q and C1 inhibitor: an atypical presentation of Waldenström macroglobulinemia
| Autor: | Bagi Jana, Anas Khanfar, Anita Trikha, Rana S. Bonds |
|---|---|
| Rok vydání: | 2013 |
| Předmět: |
medicine.medical_specialty
C1-inhibitor Diagnosis Differential immune system diseases Internal medicine Humans Medicine cardiovascular diseases Angioedema skin and connective tissue diseases Dexamethasone Aged Hematology biology business.industry Bortezomib Complement C1q food and beverages Waldenstrom macroglobulinemia medicine.disease Dermatology Phenotype Immunology Etiology biology.protein Female Rituximab Waldenstrom Macroglobulinemia medicine.symptom business Complement C1 Inhibitor Protein medicine.drug |
| Zdroj: | International Journal of Hematology. 97:654-656 |
| ISSN: | 1865-3774 0925-5710 |
| Popis: | Angioedema is a recurrent, non-pitting, non-pruritic, transitory swelling due to transient increase of endothelial permeability in the capillaries of the deep cutaneous and mucosal layers. Angioedema is generally categorized based on etiology, and characteristic lab findings are associated with each category. Cases of acquired angioedema associated with myeloproliferative disorders have been described in the literature, but these have been associated with a characteristic low C1q, a defining laboratory finding in acquired angioedema. Here we present a case of 68-year-old female with acquired angioedema that was not associated with low C1q, but was found to have Waldenström disease. Her angioedema responded dramatically to combination therapy consisting of bortezomib, rituximab, and dexamethasone. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full text from SpringerLink