Immune thrombocytopenia (ITP) in the setting of hereditary angioedema with C1 inhibitor deficiency (HAE C1INH): a possible consequence of low C4

HAE C1INH is a condition characterized by recurrent angioedema. The lack of inhibition of C1r and C1s by C1 inhibitor (C1-INH) results in low levels of C4. Previous reports have demonstrated increased risk of autoimmune disease and lymphoproliferative disorders in patients with congenital C4 deficiency. Immune thrombocytopenia (ITP) has not been previously associated with HAE C1INH.

Source: Journal of Allergy and Clinical Immunology - January 31, 2020 Category: Allergy & Immunology Authors: Abeer Hegazi, Paul Keith Source Type: research