Correspondence: Zahra Habibagahi, MD; Department of Rheumatology and Internal Medicine, Internal Medicine, Zand Street, Shiraz, Iran Tel/Fax:+98 71 36474316 Email: [email protected] Received: 29 April 2013 Revised: 8 September 2013 Accepted: 27 October 2013 Abstract Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A gen...

Hereditary angioedema (HAE) is well documented condition with inherited deficiency or dysfunction of C1 inhibitor. However, its association other immune disorders such as autoimmunity, malignancy and primary immunodeficiency not known.

BACKGROUND We report the case of a 77-year-old female with acquired angioneurotic edema, C1 esterase inhibitor level = 4mg/dL, who was scheduled to undergo laparoscopic splenectomy. METHODS In the operating room, we administered on call 500 units (UI) of C1 esterase inhibitor concentrate intravenously. Intraoperative hemodynamic instability and generalized blood oozing improved following the ...

A 52 year old woman with marginal zone lymphoma developed recurrent episodes of angio-oedema and was found to have C1 esterase inhibitor deficiency. She declined chemotherapy for the lymphoma. Fourteen months after her initial presentation she was found to be in partial remission, and this was confirmed by peripheral blood film and bone marrow examinations. This was associated with normalisatio...