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[Acquired C1 esterase inhibitor deficiency and lymphoproliferative syndromes].

Abstract
About 20 cases of acquired C1 esterase inhibitor deficiency have been reported in association with malignant lymphomas. We describe 3 such patients. The 3 patients studied were asymptomatic and had low C1q level. Danazol administration resulted in an increase of C1 esterase inhibitor in 2 patients. The complement activation in acquired C1 esterase inhibitor deficiency could be explained by interaction with pathological cells of the spleen, the blood or the bone-marrow. The mechanism of decrease of C1 INH is discussed.
AuthorsB Hory, J Panouse-Perrin, J L Dupond, R Leconte des Floris
JournalLa Revue de medecine interne (Rev Med Interne) Vol. 6 Issue 3 Pg. 266-71 (Jun 1985) ISSN: 0248-8663 [Print] France
Vernacular TitleDéficit acquis en inhibiteur de la C1 estérase et syndrome lymphoprolifératif.
PMID4048687 (Publication Type: Case Reports, English Abstract, Journal Article)
Chemical References
  • Complement C1 Inactivator Proteins
  • Complement System Proteins
  • Danazol
Topics
  • Aged
  • Complement C1 Inactivator Proteins (deficiency)
  • Complement System Proteins (analysis)
  • Danazol (therapeutic use)
  • Female
  • Humans
  • Lymphoproliferative Disorders (enzymology, immunology, therapy)
  • Male
  • Middle Aged
  • Plasmapheresis
  • Time Factors

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