C1 Esterase Inhibitor Deficiency

C1 Esterase Inhibitor Deficiency. Acquired C1 inhibitor deficiency may occur with B-cell lymphomas and some autoimmune diseases. The genetic defect is due to the heterozygous deficiency of C1-Inh that is transmitted as an autosomal dominant trait.

HAEGARDA C1 Esterase Inhibitor Subcutaneous Human is a plasma-derived concentrate of C1 Esterase Inhibitor C1-INH indicated for routine prophylaxis to prevent Hereditary Angioedema HAE attacks in patients 6 years of age and olderHAEGARDA is for subcutaneous use after reconstitution only. C1 esterase inhibitor deficiency results in hereditary angioedema. N2 - Acquired C1 esterase inhibitor deficiency is a rare condition associated with autoimmune or low-grade lymphoproliferative disorders.

Both conditions lead to recurrent angioedema that can be life threatening when the larynx is involved hereditary angioedema HAE.

C1 esterase inhibitor deficiency results in hereditary angioedema. Acquired angioedema due to deficiency of C1 esterase inhibitor also called acquired angioedema and abbreviated C1INH-AAE is a rare syndrome of recurrent episodes of angioedema without urticaria which is associated with B cell lymphoproliferative disorders in some patients Angioedema typically affects the skin or mucosal tissues of the. Adults or elderly patients are most commonly affected. This disorder can lead to.