Acquired C1 esterase inhibitor deficiency and lymphoproliferative syndromes

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....

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Veröffentlicht in:	La revue de medecine interne 1985-06, Vol.6 (3), p.266-271
Hauptverfasser:	Hory, B, Panouse-Perrin, J, Dupond, J L, Leconte des Floris, R
Format:	Artikel
Sprache:	fre
Schlagworte:	Aged Complement C1 Inactivator Proteins - deficiency Complement System Proteins - analysis Danazol - therapeutic use Female Humans Lymphoproliferative Disorders - enzymology Lymphoproliferative Disorders - immunology Lymphoproliferative Disorders - therapy Male Middle Aged Plasmapheresis Time Factors
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Zusammenfassung:	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.
ISSN:	0248-8663