Acquired Angioedema Responding to Rituximab

Acquired angioedema responding to rituximab.

Acquired angioedema: a new target for rituximab?

Acquired angioedema (AAE) is a rare disorder characterized by recurrent attacks, late onset of symptoms and a marked decrease of C1 levels. Pathogenetically it is caused by an accelerated consumption of C1-inhibitor (C1-INH), resulting in C1-INH deficiency and uncontrolled complement activation. We describe successful treatment of a patient with AEE due to C1-INH autoantibody, administering 4 w...

Acquired angioedema

Acquired angioedema (AAE) is characterized by acquired deficiency of C1 inhibitor (C1-INH), hyperactivation of the classical pathway of human complement and angioedema symptoms mediated by bradykinin released by inappropriate activation of the contact-kinin system. Angioedema recurs at unpredictable intervals, lasts from two to five days and presents with edema of the skin (face, limbs, genital...

A case of acquired angioedema associated with Waldenstrom’s macroglobulinemia treated with rituximab

Case presentation We report a case of Waldenstrom ́s macroglobulinemia causing an acquired deficiency of C1 esterase inhibitor in a 40-year-old woman. She initially presented with an episode of angioedema followed by many episodes of abdominal distention associated with pain, vomiting, and diarrhea for 1.5 years. Work-up revealed low C1 esterase inhibitor levels, normal C3, and nonexistent C4. A...

Rituximab therapy in a patient with low grade B-cell lymphoproliferative disease and concomitant acquired angioedema

Acquired angioedema is often associated with significant morbidity. An underlying lymphatic malignancy, autoimmune disorder, adenocarcinoma, or other malignancy may be present. Screening for these disorders should occur in all patients with acquired angioedema as treatment may result in resolution of angioedema.