Acquired C1-inhibitor deficiency in essential cryoglobulinemia and macrocryoglobulinemia.

Abstract:

:In 5 patients with mixed cryoglobulinemia, 3 with Waldenström's disease and 2 with essential cryoglobulinemia, a C1-inhibitor (C1-INH) deficiency was discovered. The complement profile was characteristic of the acquired type: the total hemolytic activity and the early components were reduced, C3 was diminished in 1 patient only, C5 and C9 were normal or elevated. 1 atient with Waldenström's disease and 1 with essential cryoglobulinemia experienced episodes of angioedema. Circulating immune complexes were found in all patients' sera by the 125I-radiolabelled C1q binding activity (C1q BA) test. The values of the C1q binding activity were correlated with the depletion of the early complement components and that of C1-inh. 4 patients showed circulating 7S IgM. Our data support the hypothesis of a complement activation by the cryoprecipitating immune complexes; the C1-INH depletion is secondary to its consumption following C1 activation.

journal_name

Acta Haematol

journal_title

Acta haematologica

authors

Casali P,Borzini P,Pioltelli P,Invernizzi F,Zanussi C

doi

10.1159/000207773

subject

Has Abstract

pub_date

1978-01-01 00:00:00

pages

277-84

issue

5

eissn

0001-5792

issn

1421-9662

journal_volume

59

pub_type

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