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Development of immunologic functions after bone marrow transplantation in
33 patients with severe combined immunodeficiency
L Wijnaendts, F Le Deist, C Griscelli and A Fischer
INSERM U 132, Hopital des Enfants-Malades, Paris, France.
We retrospectively analyzed the development of lymphocytes and of the main
immunological functions in 33 patients with severe combined
immunodeficiency who survived at least 6 months after bone marrow
transplantation (BMT). Eighteen patients received HLA-identical BM and 15
received HLA-nonidentical BM. Development of immune functions occurred
faster after HLA-identical BMT as full T- and B-lymphocyte- mediated
responses were present at day 186 versus 505, respectively (P = .05). In
addition, antibody responses remain completely or partially absent in 8 of
15 patients of the second group. Detection of antibody response after
HLA-incompatible BMT correlated with engraftment of donor B cells in
informative cases. In patients who received an HLA- nonidentical BMT after
chemotherapy (6 of 15), development of immune functions occurred more
rapidly and 6 of 6 had B-cell functions, including normal antibody
production. Autoimmunity was not uncommon and was found after
HLA-incompatible BMT (4 of 15) or after HLA-partially phenotypically
identical BMT (2 of 3). Antibodies were in most cases specific for blood
cells. Occurrence of autoimmunity correlates with poor B-cell functions and
to a lesser extent with defective T-cell responses. This type of study may
lead to definition of a more accurate strategy for performing BMT in
patients with severe combined immunodeficiency.
Volume 74,
Issue 6,
pp. 2212-2219,
11/01/1989
Copyright © 1989 by The American Society of Hematology
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