| Lymphoma-associated paraneoplastic angioedema with normal C1-inhibitor activity: does danazol work? | |
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MedLine Citation:
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PMID: 15495244 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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S Gaur; J Cooley; L Aish; R Weinstein |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: American journal of hematology Volume: 77 ISSN: 0361-8609 ISO Abbreviation: Am. J. Hematol. Publication Date: 2004 Nov |
Date Detail:
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Created Date: 2004-10-25 Completed Date: 2005-02-03 Revised Date: 2007-11-15 |
Medline Journal Info:
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Nlm Unique ID: 7610369 Medline TA: Am J Hematol Country: United States |
Other Details:
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Languages: eng Pagination: 296-8 Citation Subset: IM |
Affiliation:
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Department of Medicine, Caritas St. Elizabeth's Medical Center, Tufts University School of Medicine, Boston, Massachusetts 02135, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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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:
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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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