Original articleAcquired deficiency of the inhibitor of the first component of complement: Report of five additional cases with commentary on the syndrome☆
References (29)
- et al.
Acquired C1 deficiency in lymphosarcoma
Clin Immunol Immunopathol
(1972) - et al.
A biochemical abnormality in hereditary angioneurotic edema: absence of serum inhibitor of C'1 esterase
Am J Med
(1963) - et al.
Acquired angioedema with lymphoproliferative disorder: association of C1 inhibitor deficiency with cellular abnormality
Blood
(1976) - et al.
Acquired angioedema associated with rectal carcinoma and its response to danazol therapy
J Allergy Clin Immunol
(1978) - et al.
Expression of human B cell-associated antigens on leukemias and lymphomas: a model of human B cell differentiation
Blood
(1984) - et al.
Clinical/biochemical assessment of acquired CINH deficiency
J Allergy Clin Immunol
(1983) - et al.
Clinical and biochemical effects of stanozolol therapy for hereditary angioedema
J Allergy Clin Immunol
(1981) - et al.
Action of complement in hereditary angioneurotic edema: the role of C'1 esterase
J Clin Invest
(1964) - et al.
Natural control mechanisms of the complement system
- et al.
Evidence for immune complexes involving antilymphocyte antibodies associated with hypocomplementemia in chronic lymphocytic leukemia (CLL)
Clin Exp Immunol
(1976)
Angioedema and hypocomplementemia
J Allergy Clin Immunol
Acquired C1 inhibitor deficiencies in lymphoproliferative diseases with serum immunoglobulin abnormalities: a study of three cases
Blut
Treatment of acquired C1 inhibitor deficiency with danazol
Ann Intern Med
Diffuse normolipaemic plane xanthomatosis, an abnormal complement component profile
Clin Exp Immunol
Cited by (62)
Chapter 24 Danazol Therapy
2008, Handbook of Systemic Autoimmune DiseasesCitation Excerpt :These complexes were elevated before treatment, but reverted rapidly to normal values during androgen therapy and remained normal with reduction of dose as long as the patient remained free of symptoms. In addition, attenuated androgens have been shown to prevent symptoms and increase C1-INH plasma levels in non-autoantibody-mediated acquired angioedema (Sheffer et al., 1985). Danazol has been useful in treating other immune-mediated diseases such as Henoch–Schönlein purpura (Lee et al., 1993).
Acquired Deficiency of the Inhibitor of the First Complement Component: Presentation, Diagnosis, Course, and Conventional Management
2006, Immunology and Allergy Clinics of North AmericaCitation Excerpt :Successful treatment of the underlying disease has been shown to resolve angioedema symptoms and biochemical abnormalities in patients who have acquired C1-INH deficiency. The first such report from Cohen and colleagues [32] has been repeatedly confirmed [4,8,27,33–39], although the details of the responses have demonstrated a wide range of variability. Disappearance of angioedema may be temporary, even without evidence of a relapse of the associated disease [32,35].
A patient with facial and neck swelling, dyspnea, and dysphagia
2005, Annals of Allergy, Asthma and ImmunologyHereditary and acquired angioedema: Problems and progress: Proceedings of the third C1 esterase inhibitor deficiency workshop and beyond
2004, Journal of Allergy and Clinical ImmunologyCitation Excerpt :Like other acquired forms of protein deficiencies, the course of acquired C1-INH deficiency can be related to the course of the underlying disease. The possibility of reversing the biochemical and/or clinical abnormalities of acquired C1-INH deficiency by curing the associated disease was reported by Cohen et al105 and subsequently confirmed.122,132,370-372 However, the response can be temporary, even without evidence of relapse of the associated disease.373
Clinical and laboratory evaluation of complement deficiency
2004, Journal of Allergy and Clinical ImmunologyCitation Excerpt :Goodpasture's syndrome is another well-studied example of this general paradigm. Acquired angioedema (AAE) is caused by clearance of C1-Inh, either through excessive activation of C1 or other enzymes that react with it or by autoantibodies to the C1-Inh.55–57 Even though its clinical features are indistinguishable from those of HAE, factors such as lack of family history, later age of onset, and the presence of a malignancy (in ∼50%) favor AAE over HAE.40,58
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Supported by grants AI-22531, AI-10356, AI-17917, HL-17382, AM-05577, RR-02172, and RR-05669 from the National Institutes of Health.