title: Studies on lymphocyte characteristics in patients with homozygous alpha 1-proteinase inhibitor deficiency during substitution therapy.
abstract: Alpha 1-proteinase inhibitor (alpha 1-PI) has been demonstrated to suppress mitogen-induced lymphocyte response in vitro. To evaluate the effect of intravenous application of human alpha 1-PI (Prolastin HS) on cellular immunity, we determined total lymphocyte count, lymphocyte subsets and lymphocyte response to concanavalin A, before and 24 h after infusion of 60 mg.kg-1 body weight alpha 1-PI in eight patients with homozygous alpha 1-PI deficiency (PiZ phenotype). The results were compared with two blood samples from seven healthy controls. After infusion, serum alpha 1-PI levels were increased from 0.98 +/- 0.24 to 2.68 +/- 0.51 g.l-1. No significant differences were found for total lymphocyte count, lymphocyte subsets and lymphocyte response between both groups in both samples. Maximum 3H-thymidine incorporation before and after infusion showed no significant difference; the same was true for the two control samples. However, additional incubation in vitro with alpha 1-PI 5 g.l-1 led to a significant (p < 0.03) decrease of lymphocyte proliferation in samples after infusion. Our data indicate that alpha 1-PI substitution therapy does not lead to a major suppression of lymphocyte response to concanavalin A in PiZ individuals in vivo, although a suppressive effect was found after additional in vitro incubation with alpha 1-PI.
authors.affiliation: Dept of Pulmonary Medicine II, Chest Hospital Heckeshorn, Berlin, Germany.
authors.forename: N
authors.initials: N
authors.lastname: Schoenfeld
authors.forename: M
authors.initials: M
authors.lastname: Schmitt
authors.forename: N
authors.initials: N
authors.lastname: Remy
authors.forename: U
authors.initials: U
authors.lastname: Wahn
authors.forename: R
authors.initials: R
authors.lastname: Loddenkemper
mesh_terms: D006720:Homozygote; D006801:Humans; D008213:Lymphocyte Activation; D016131:Lymphocyte Subsets; D010641:Phenotype; D000515:alpha 1-Antitrypsin / Q000235:genetics / Q000627:therapeutic use*; D019896:alpha 1-Antitrypsin Deficiency
chemical_list: D000515:alpha 1-Antitrypsin