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Immunol Allergy Clin North Am. 2006 Nov;26(4):669-90.

Acquired deficiency of the inhibitor of the first complement component: presentation, diagnosis, course, and conventional management.

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1
Department of Internal Medicine, San Giuseppe Hospital-AFaR (Ospedale San Giuseppe), University of Milan, Via San Vittore 12, 20123 Milano, Italy.

Abstract

Acquired deficiency of the inhibitor of the first complement component (C1-INH) is a rare, potentially life-threatening disease whose cause, course, and management are not completely defined. This article analyzes the etiopathogenetic mechanism, the clinical presentation, and the relationship between acquired C1-INH deficiency and lymphoproliferative disorders. Moreover, the authors give an overview of the outcome of the disease and the different therapies proposed to cure it.

PMID:
17085284
DOI:
10.1016/j.iac.2006.08.002
[Indexed for MEDLINE]
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