Document Detail


Lymphoma-associated paraneoplastic angioedema with normal C1-inhibitor activity: does danazol work?
MedLine Citation:
PMID:  15495244     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A patient with splenic marginal zone lymphoma presented with severe, symptomatic acquired angioedema. Unlike previously reported cases, his serum levels of complement and C1 inhibitor (C1-INH) were not decreased. Nonetheless, he responded clinically to treatment with an attenuated androgen and, after therapeutic splenectomy, has been maintained asymptomatic without androgen therapy for 5 years. Thus stimulation of C1-INH synthesis may overcome paraneoplastic angioedema in patients with lymphoproliferative disorders despite the absence of typical evidence for a quantitative or qualitative defect in C1-INH. Androgens should not be withheld despite a normal level of C1-INH and complement in symptomatic patients.
Authors:
S Gaur; J Cooley; L Aish; R Weinstein
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of hematology     Volume:  77     ISSN:  0361-8609     ISO Abbreviation:  Am. J. Hematol.     Publication Date:  2004 Nov 
Date Detail:
Created Date:  2004-10-25     Completed Date:  2005-02-03     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  7610369     Medline TA:  Am J Hematol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  296-8     Citation Subset:  IM    
Affiliation:
Department of Medicine, Caritas St. Elizabeth's Medical Center, Tufts University School of Medicine, Boston, Massachusetts 02135, USA.
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MeSH Terms
Descriptor/Qualifier:
Adult
Angioedema / drug therapy*,  etiology,  immunology*,  pathology
Complement C1 Inactivator Proteins / biosynthesis*
Danazol / therapeutic use*
Estrogen Antagonists / therapeutic use
Humans
Lymphoma, B-Cell, Marginal Zone / complications*,  immunology,  pathology
Male
Splenectomy
Splenic Neoplasms / complications*,  immunology,  pathology
Treatment Outcome
Chemical
Reg. No./Substance:
0/Complement C1 Inactivator Proteins; 0/Estrogen Antagonists; 17230-88-5/Danazol

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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