Acquired C1 inhibitor (C1-INH) deficiency type II. Replacement therapy with C1-INH and analysis of patients' C1-INH and anti-C1-INH autoantibodies.
نویسندگان
چکیده
منابع مشابه
Use of a C1 Inhibitor Concentrate in Adults ≥65 Years of Age with Hereditary Angioedema: Findings from the International Berinert® (C1-INH) Registry
BACKGROUND Treatment of hereditary angioedema (HAE) in 'older adults' (those aged ≥65 years) has not been well studied. The international Berinert Patient Registry collected data on the use of intravenous plasma-derived, pasteurized, nanofiltered C1-inhibitor concentrate (pnfC1-INH; Berinert®/CSL Behring) in patients of any age, including many older adults. METHODS This observational registry...
متن کاملLymphoproliferative disease and acquired C1 inhibitor deficiency.
Angioedema due to acquired deficiency of the C1-inhibitor is a bridging condition between autoimmunity and lymphoproliferation. We report 32 patients with acquired C1 inhibitor deficiency: 23 have anti C1-inhibitor autoantibodies; 13 have monoclonal gammopathies of unknown significance and 9 have non-Hodgkin's lymphoma. Our series suggest that different forms of B cell disorders coexist and/or ...
متن کاملAcquired C 1 Inhibitor ( Cl - INH ) Deficiency Type
The response of two patients with autoantibody-mediated Clinhibitor (Cl-INH) deficiency to replacement therapy with Cl-INH was studied over a period of 3 d. In patient 1 an acute attack of angioedema was successfully managed by infusion of 1,000 U ofC1-INH concentrate. C1-INH function returned to normal levels within 30 min., while CH50 and C4 peaked after 6-7 h and Cl hemolytic activity reache...
متن کاملCLINICAL REVIEW Acquired C1 Esterase Inhibitor Deficiency
Acquired C1 esterase inhibitor deficiency is a rare condition associated with autoimmune or low-grade lymphoproliferative disorders. Adults or elderly patients are most commonly affected. The diagnosis is suspected when patients present with recurrent angioedema and low serum levels of C4 with normal levels of C3. Low levels of C1q and low C1 esterase inhibitor activity confirm the diagnosis. I...
متن کاملPlasma levels of C1- inhibitor complexes and cleaved C1- inhibitor in patients with hereditary angioneurotic edema.
C1- inhibitor (C1(-)-Inh) catabolism in plasma of patients with hereditary angioneurotic edema (HANE) was assessed by measuring the complexes formed by C1(-)-Inh with its target proteases (C1-s, Factor XIIa, and kallikrein) and a modified (cleaved) inactive form of C1(-)-Inh (iC1(-)-Inh). This study was performed in plasma from 18 healthy subjects and 30 patients with HANE in remission: 20 with...
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ژورنال
عنوان ژورنال: Journal of Clinical Investigation
سال: 1989
ISSN: 0021-9738
DOI: 10.1172/jci114084