Treatment of pure red cell aplasia with ciclosporin: suppression of activated T suppressor/cytotoxic and NK-like cells in marrow and blood correlates with haematological response

Eur J Haematol. 1988 Sep;41(3):204-11. doi: 10.1111/j.1600-0609.1988.tb01182.x.


In pure red cell aplasia (PRCA), suppression of erythropoiesis is most probably effected by cellular and/or antibody-mediated immune mechanisms. We have prospectively investigated the patterns of activated T-lymphocyte subsets and natural killer (NK)-like cells in the bone marrow (BM) and peripheral blood (PB) of 6 PRCA patients prior to and during treatment with the T-cell selective immunosuppressive drug Ciclosporin (CS; Cyclosporin-A). Before CS therapy, large proportions of circulating activated HLA-DR+ T-suppressor/cytotoxic cells (28%, 11-41; median and range), DR+ T-helper cells (7%, 4-13) and cytoplasmic interferon-gamma+ (IFN-gamma) lymphocytes (20%, 9-33) were found in PRCA, but were barely detectable in healthy controls. Similarly, high levels of activated T cells were present in patient marrows. Initiation of CS therapy resulted in rapidly decreasing levels of activated T cells and NK cells both in PB and in BM. During follow-up of individual patients, an inverse relationship was observed between the haematological response to CS and the levels of activated T-suppressor/cytotoxic cells, IFN-gamma+ lymphocytes (in PB and BM) and NK-like cells (in BM). The present correlations indicate a pathogenetic role of phenotypically activated T-suppressor/cytotoxic cells and possibly NK-like cells in PRCA. During successful treatment with CS, these cell types are reduced in numbers but reappear in relapse. Our results also pinpoint the clinical value of monitoring activated T-cell subsets for the prediction of immunological remission in PRCA and related disorders.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Biomarkers
  • Bone Marrow / drug effects
  • Bone Marrow / pathology
  • Cyclosporins / therapeutic use*
  • Female
  • Hematopoiesis / drug effects*
  • Humans
  • Killer Cells, Natural / drug effects*
  • Killer Cells, Natural / immunology
  • Lymphocyte Activation / drug effects*
  • Male
  • Middle Aged
  • Phenotype
  • Prospective Studies
  • Red-Cell Aplasia, Pure / blood
  • Red-Cell Aplasia, Pure / drug therapy*
  • Red-Cell Aplasia, Pure / pathology
  • T-Lymphocytes / classification
  • T-Lymphocytes, Cytotoxic / drug effects*
  • T-Lymphocytes, Cytotoxic / immunology


  • Biomarkers
  • Cyclosporins