Skip to main content

Advertising Disclaimer »

Main menu

  • Journals
    • Pediatrics
    • Hospital Pediatrics
    • Pediatrics in Review
    • NeoReviews
    • AAP Grand Rounds
    • AAP News
  • Authors/Reviewers
    • Submit Manuscript
    • Author Guidelines
    • Reviewer Guidelines
    • Open Access
    • Editorial Policies
  • Content
    • Current Issue
    • Online First
    • Archive
    • Blogs
    • Topic/Program Collections
    • NCE Meeting Abstracts
  • AAP Policy
  • Supplements
  • Multimedia
  • Subscribe
  • Alerts
  • Careers
  • Other Publications
    • American Academy of Pediatrics

User menu

  • Log in

Search

  • Advanced search
American Academy of Pediatrics

AAP Gateway

Advanced Search

AAP Logo

  • Log in
  • Journals
    • Pediatrics
    • Hospital Pediatrics
    • Pediatrics in Review
    • NeoReviews
    • AAP Grand Rounds
    • AAP News
  • Authors/Reviewers
    • Submit Manuscript
    • Author Guidelines
    • Reviewer Guidelines
    • Open Access
    • Editorial Policies
  • Content
    • Current Issue
    • Online First
    • Archive
    • Blogs
    • Topic/Program Collections
    • NCE Meeting Abstracts
  • AAP Policy
  • Supplements
  • Multimedia
  • Subscribe
  • Alerts
  • Careers
American Academy of Pediatrics
INFECTIOUS DISEASE AND IMMUNOLOGY: IMMUNODEFICIENCY DISEASES

PREVALENCE OF MONOCLONAL GAMMOPATHY IN PATIENTS PRESENTING WITH ACQUIRED ANGIOEDEMA TYPE 2

Allen Adinoff
Pediatrics August 2003, 112 (Supplement 2) 490;
Allen Adinoff
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Info & Metrics
  • Comments
Loading
Download PDF

Fremeaux-Bacchi V, Guinnepain MT, Cacoub P, et al. Am J Med. 2002;113:194–199

Purpose of the Study.

Acquired angioedema type 1 is characterized by a C1 inhibitor deficiency in patients with lymphoproliferative disorders, whereas acquired angioedema type 2 is characterized by anti-C1 inhibitor antibodies, and has not been thought to be associated with lymphoproliferative disease. We studied the clinical features, complement profiles, and associated diseases in 19 new patients with diagnosed acquired angioedema type 2.

Study Population and Methods.

Plasma concentrations and functional activity of complement components were measured by conventional techniques. Functional C1 inhibitor activity was assessed by a chromogenic assay. Autoantibodies to C1 inhibitor were detected using an enzyme-linked immunosorbent assay.

Results.

The 11 men and 8 women (median age: 60 years) presented with recurrent attacks of angioedema. All patients had detectable anti-C1 inhibitor antibodies in serum. A monoclonal gammopathy was detected in 12 patients (63%) at the time of diagnosis, 11 of whom had an immunoglobulin peak of the same heavy- and light-chain isotypes as the acquired anti-C1 inhibitor antibody. Three of these 12 patients developed a malignant lymphoproliferative disease.

Conclusions.

As with type 1 disease, a large proportion of patients with acquired angioedema type 2 have a lymphoproliferative disorder.

Reviewer’s Comments.

These disorders present only rarely, so I’m always having to go back and refresh my memory. However, unlike the acquired chronic urticarias, the acquired C1 inhibitor deficiency syndromes are commonly associated with lymphoproliferative disorders, so we need to pursue things pretty aggressively. Don’t be afraid to consult your friendly oncologist.

View Abstract
PreviousNext
Back to top

Advertising Disclaimer »

In this issue

Pediatrics
Vol. 112, Issue Supplement 2
1 Aug 2003
  • Table of Contents
  • Index by author
View this article with LENS
PreviousNext
Email Article

Thank you for your interest in spreading the word on American Academy of Pediatrics.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
PREVALENCE OF MONOCLONAL GAMMOPATHY IN PATIENTS PRESENTING WITH ACQUIRED ANGIOEDEMA TYPE 2
(Your Name) has sent you a message from American Academy of Pediatrics
(Your Name) thought you would like to see the American Academy of Pediatrics web site.
Request Permissions
Article Alerts
Sign In to Email Alerts with your Email Address
Citation Tools
PREVALENCE OF MONOCLONAL GAMMOPATHY IN PATIENTS PRESENTING WITH ACQUIRED ANGIOEDEMA TYPE 2
Allen Adinoff
Pediatrics Aug 2003, 112 (Supplement 2) 490;

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Share
PREVALENCE OF MONOCLONAL GAMMOPATHY IN PATIENTS PRESENTING WITH ACQUIRED ANGIOEDEMA TYPE 2
Allen Adinoff
Pediatrics Aug 2003, 112 (Supplement 2) 490;
del.icio.us logo Digg logo Reddit logo Twitter logo CiteULike logo Facebook logo Google logo Mendeley logo
Print
Download PDF
Insight Alerts
  • Table of Contents

Jump to section

  • Article
    • Purpose of the Study.
    • Study Population and Methods.
    • Results.
    • Conclusions.
    • Reviewer’s Comments.
  • Info & Metrics
  • Comments

Related Articles

  • No related articles found.
  • Google Scholar

Cited By...

  • No citing articles found.
  • Google Scholar

More in this TOC Section

INFECTIOUS DISEASE AND IMMUNOLOGY: IMMUNODEFICIENCY DISEASES

  • CUTANEOUS MANIFESTATIONS OF HYPER-IgE SYNDROME IN INFANTS AND CHILDREN
  • CLINICAL FINDINGS LEADING TO THE DIAGNOSIS OF X-LINKED AGAMMAGLOBULINEMIA
Show more INFECTIOUS DISEASE AND IMMUNOLOGY: IMMUNODEFICIENCY DISEASES

ABSTRACTS

  • CUTANEOUS MANIFESTATIONS OF HYPER-IgE SYNDROME IN INFANTS AND CHILDREN
  • CLINICAL FINDINGS LEADING TO THE DIAGNOSIS OF X-LINKED AGAMMAGLOBULINEMIA
  • THE ROLE OF ANTICHOLINERGICS IN ACUTE ASTHMA TREATMENT
Show more ABSTRACTS

Similar Articles

Subjects

  • Infectious Disease
    • Infectious Disease
  • Journal Info
  • Editorial Board
  • Editorial Policies
  • Overview
  • Licensing Information
  • Authors/Reviewers
  • Author Guidelines
  • Submit My Manuscript
  • Open Access
  • Reviewer Guidelines
  • Librarians
  • Usage Stats
  • Support
  • Contact Us
  • Subscribe
  • About
  • International Access
  • Terms of Use
  • Privacy Statement
  • FAQ
  • RSS Feeds
  • AAP.org
  • shopAAP
  • Follow American Academy of Pediatrics on Instagram
  • Visit American Academy of Pediatrics on Facebook
  • Follow American Academy of Pediatrics on Twitter
  • Follow American Academy of Pediatrics on Youtube
  • RSS
American Academy of Pediatrics

© 2019 American Academy of Pediatrics