Acquired CI-inhibitor deficiency in essential cryoglobulinemia and macrocryoglobulinemia

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

Research output: Contribution to journalArticle

20 Scopus citations

Abstract

In 5 patients with mixed cryoglobulinemia, 3 with Waldenström’s disease and 2 with essential cryoglobulinemia, a Cl-inhibitor (CI-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 patient with Waldenstrom’s disease and 1 with essential cryoglobulinemia experienced episodes of angioedema. Circulating immune complexes were found in all patients’ sera by the 12!I-radio-labelled Clq binding activity (Clq BA) test. The values of the Clq binding activity were correlated with the depletion of the early complement components and that of CI-INH. 4 patients showed circulating 7S IgM. Our data support the hypothesis of a complement activation by the cryoprecipitating immune complexes; the CI-INH depletion is secondary to its consumption following Cl activation.

Original languageEnglish (US)
Pages (from-to)277-284
Number of pages8
JournalActa Haematologica
Volume59
Issue number5
DOIs
StatePublished - Jan 1 1978
Externally publishedYes

Keywords

  • <sup>125</sup>I Clq binding activity
  • Acquired CI-inhibitor deficiency
  • Complement
  • Essential cryoglobulinemia
  • Immune complexes
  • Waldenstöm’s disease

ASJC Scopus subject areas

  • Hematology

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