title: Lymphocyte characteristics and function in paroxysmal nocturnal haemoglobinuria.
abstract: 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.
mesh_terms: D000911:Antibodies, Monoclonal / Q000276:immunology; D003208:Concanavalin A / Q000494:pharmacology; D006457:Hemoglobinuria, Paroxysmal / Q000276:immunology*; D006801:Humans; D007204:Indium; D015125:Oxyquinoline / Q000378:metabolism; D011868:Radioisotopes; D013601:T-Lymphocytes / Q000145:classification / Q000276:immunology*; D006377:T-Lymphocytes, Helper-Inducer / Q000145:classification; D050378:T-Lymphocytes, Regulatory / Q000145:classification
chemical_list: D000911:Antibodies, Monoclonal; D011868:Radioisotopes; D007204:Indium; D003208:Concanavalin A; D015125:Oxyquinoline