Transplacentally acquired maternal T lymphocytes in severe combined immunodeficiency: a study of 121 patients.

Abstract:

:A study in 121 infants with severe combined immunodeficiency (SCID) was performed to determine the prevalence of an engraftment by transplacentally acquired maternal T cells and to explore clinical and immunological findings related to this abnormality. Each newly diagnosed patient with SCID presenting with circulating T cells was evaluated for chimerism by performing selective HLA typing of T cells and non-T cells. In patients with engraftment, maternal T cells were characterized phenotypically and functionally, and results were correlated with clinical findings in the patients. Maternal T cells were detected in the circulation in 48 patients; these cells ranged from fewer than 100/microL in 14 cases to more than 2000/microL in 4 cases (median, 415/microL). Clinical signs of graft-versus-host disease (GVHD) were absent in 29 patients. In the other cases, manifestations of GVHD were present, involving the skin and in 14 cases also the liver. Skin GVHD was mild in 8 patients. In these patients, as well as in patients with no signs of GVHD, maternal T cells were predominantly CD8(+) and, with one exception, failed to respond to mitogen stimulation. In 9 patients, manifestations of skin GVHD were prominent. T cells in these cases were predominantly CD4(+) and responded, with one exception, to mitogen stimulation. In 8 of the cases with prominent skin GVHD, the underlying SCID variant was characterized by the absence of B cells. In this study, further understanding is provided of a phenomenon that is responsible for the significant heterogeneity of clinical and immunological findings in SCID.

journal_name

Blood

journal_title

Blood

authors

Müller SM,Ege M,Pottharst A,Schulz AS,Schwarz K,Friedrich W

doi

10.1182/blood.v98.6.1847

subject

Has Abstract

pub_date

2001-09-15 00:00:00

pages

1847-51

issue

6

eissn

0006-4971

issn

1528-0020

journal_volume

98

pub_type

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