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Blood, 15 September 2001, Vol. 98, No. 6, pp. 1847-1851
IMMUNOBIOLOGY
Transplacentally acquired maternal T lymphocytes in
severe combined immunodeficiency: a study of 121 patients
Susanna M. Müller,
Markus Ege,
Annette Pottharst,
Ansgar S. Schulz,
Klaus Schwarz, and
Wilhelm Friedrich
From the Department of Pediatrics and the Department of
Transfusion Medicine, University of Ulm, Germany.
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/µL in 14 cases to more than 2000/µL in 4 cases
(median, 415/µL). 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.
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