Acquired angioedema due to C1-INH deficiency (C1-INH-AAE) can occur when there are acquired (not inherited) deficiencies of C1-INH.
A quantitative or functional C1-INH deficiency with negative family history and low C1q is diagnostic of C1-INH-AAE.
The most common conditions associated with C1-INH-AAE are:
- B-cell lymphoproliferative disorders
A diagnosis of C1-INH-AAE can precede a diagnosis of lymphoproliferative disease and confers an increased risk for developing non-Hodgkin lymphoma.
Treatment focuses on symptom control with:
- therapies that regulate bradykinin activity (C1-INH concentrate, icatibant, ecallantide, tranexamic acid, androgens)
- treatment of any underlying conditions
Acquired C1 Inhibitor Deficiency http://buff.ly/2sx8r8B