Serum Immunoglobulins and Lymphocyte Subpopulations Derangement in Turner's Syndrome

Abstract

SUMMARY Abnormalities of the proportions of peripheral blood lymphocyte subpopulations and of immunoglobulin serum levels were found in twenty patients affected by Turner's syndrome. A slight but significantly decreased percentage of circulating T and B cells, an increased percentage of null cells and a decreased in vitro, responsiveness of lymphocytes to phytohaemagglutinin, concanavalin A and pokeweed mitogen were found in Turner's syndrome patients. IgG serum level was found significantly decreased in comparison with age‐matched fifty‐seven normal males and fifty‐seven normal females and IgM serum level was intermediate between female and male values; Turner's syndrome patients with monosomy had an IgM serum concentration very close to male values. The derangement of T and B lymphocyte subpopulations, probably related to the aneuploidy, does not seem to be a severe one but it could account for the immunoglobulin abnormalities and for the association of Turner's syndrome with immunological disorders such as autoimmune diseases. The role of X chromosome on IgM serum level is discussed.

Increased spontaneous, tumor necrosis factor receptor- and CD95 (Fas)-mediated apoptosis in cord blood T-cell subsets from Turner's syndrome

“…11,[12][13][14][15][16] In the present neonate with Turner's syndrome, we have also observed decreased proportions of both CD4+ (7.6%) and CD8+ (5.2%) T cells as compared to normal cord blood CD4+ (41.9%) and CD8+ (30.8%) T cells ( Figure 1). However, the mechanism(s) of cell-mediated immune defects and T-cell lymphopenia in Turner's syndrome has not been explored.…”

“…In Turner's syndrome, cell-mediated defects include decreased proliferative response to mitogens, recall antigens, and autoantigens, 11,12,16 decreased expression of IL-2 receptors, decreased expression of costimulatory molecules on CD4+ T cells, and CD4+ lymphopenia and low CD4+/CD8+ T-cell ratios. 11,[12][13][14][15][16] In the present neonate with Turner's syndrome, we have also observed decreased proportions of both CD4+ (7.6%) and CD8+ (5.2%) T cells as compared to normal cord blood CD4+ (41.9%) and CD8+ (30.8%) T cells ( Figure 1).…”

“…[9][10][11][12][13][14][15][16][17] However, the mechanism(s) for the underlying immunodeficiency has not been explored.…”

Allogeneic stem cell transplantation from donors with mosaic Turner syndrome

“…3 Various immunological disturbances have been detected in patients with TS, including decreased numbers of circulating T-lymphocytes. 2,4 Consequently, the study of immune recovery of the donor's 45,X T cells following SCT is particularly interesting, because it might influence infectious complications, antitumor activity, the development and the extent of acute and chronic GVHD, as well as the process of engraftment of hematopoietic stem cells. It is also not clear if 45,X hematopoietic stem cells would respond normally to immunosuppression.…”

Heterogeneous loss of the Y chromosome in leukocyte lineages of donor origin after stem cell transplantation

“…In fact, it has been shown in Turner syndrome patients show various immunological disorders. [5][6][7] Moreover, monosomy X in T cells would be related to an increased spontaneous as well as death receptor-meditated apoptosis. 8 In addition, monosomy X T cells and monocytes of these patients showed a decreased sensitivity to insulin-like growth factor I (IGF-1) and growth hormone, and therefore a decreased monocyte-stimulated T lymphocyte proliferation.…”