concanavalin-a and Hemoglobinuria--Paroxysmal

In six patients with paroxysmal nocturnal haemoglobinuria (PNH) lymphocyte studies were performed to investigate whether the observed immunological dysfunction could be ascribed to a defect in lymphocytes as result of the PNH characteristics, or to an imbalance between T-cell subsets. The PNH characteristics were studied by means of the effect of serum and acidified serum on Indium111-oxine labelled lymphocytes. No increase in release of Indium111-oxine was found when lymphocytes were exposed to acidified sera. Thus a complement mediated lymphocyte lysis in PNH could not be demonstrated. T-cells were defined by monoclonal antibodies, directed at total T-cells (OKT3), helper T cells (OKT4) and suppressor/cytotoxic T-cells (OKT8). In two of the six patients a decreased proportion of OKT3 cells was found, while a significantly depressed ratio of OKT4/OKT8 cells was present in the whole group. No obvious correlation was found between a functional assay - the concanavalin-A suppressor cell activity - and the ratio of OKT4/OKT8 positive cells. It is concluded that the PNH characteristics could not be demonstrated in the lymphocytes; and that the immunological dysregulation in PNH may be ascribed to an imbalance of T-cell subsets, while a decreased number of monocytes, defined by the monoclonal antibody OKM1, may contribute to this defect.

Topics: Antibodies, Monoclonal; Concanavalin A; Hemoglobinuria, Paroxysmal; Humans; Indium; Oxyquinoline; Radioisotopes; T-Lymphocytes; T-Lymphocytes, Helper-Inducer; T-Lymphocytes, Regulatory

Topics: ABO Blood-Group System; Acetamides; Animals; beta-Aminoethyl Isothiourea; Binding Sites, Antibody; Cell Line; Cell Membrane; Cell Separation; Complement System Proteins; Concanavalin A; Cysteine; Cytotoxicity Tests, Immunologic; Dithiothreitol; Dose-Response Relationship, Drug; Erythrocytes; Glutathione; Hemagglutination Tests; Hemoglobinuria, Paroxysmal; Humans; Hydrogen-Ion Concentration; Hypersplenism; Immune Adherence Reaction; Isoantibodies; Leukemia, Lymphoid; Lupus Erythematosus, Systemic; Lymphocytes; Mercaptoethanol; Microscopy, Electron, Scanning; Sheep; Sulfhydryl Compounds