Immunoreconstitution by peripheral blood leukocytes in adenosine deaminase-deficient severe combined immunodeficiency

K. C. Rich, Carol M Richman, E. Mejias, P. Daddona

Research output: Contribution to journalArticle

14 Citations (Scopus)

Abstract

Transplantation of histocompatible allogeneic peripheral blood leukocytes resulted in successful reconstitution of an adenosine deaminase (ADA)-deficient, severe combined immune-deficient patient. Erythrocyte transfusions before the transplant were associated with a rise of serum immunoglobulin concentration to normal without improvement in T cell function. The patient received 5 x 107 peripheral blood mononuclear leukocytes/kg obtained from the histocompatible father by leukopheresis. Three weeks after the transplant the lymphocyte count, proportion of E rosetting lymphocytes, and the ADA content of the patient's mononuclear leukocytes became normal while the phytohemagglutinin-stimulated blastogenic responses improved and became normal 52 days after the transplant. Antibody response to diphtheria immunization and response to naturally acquired herpes simplex infection were normal while isohemagglutinins progressively increased. Immunization with a neoantigen, bacteriophage ∅X 174, resulted in a small but definite antibody response but no amplification of the response after secondary immunization. A positive reaction to a skin test for Candida albicans developed. Erythrocyte deoxy ATP (dATP) concentration decreased during the course of erythrocyte transfusions. Nine months after the transplant, the erythrocyte dATP was elevated to twice pretransfusion levels while mononuclear leukocyte dATP varied from normal to elevated during the first 4 mth of the posttransplant period, but remained normal during the last 8 mth. The improvement in immune function persisted during the 12 mth posttransplant observation period while the mononuclear leukocyte ADA concentration stabilized at ~0.25 of normal, which is similar to the enzyme activity of the donor cells. This in vivo study supports the hypothesis that lymphoid precursor cells are present in peripheral blood which may partially reconstitute an immune-deficient recipient.

Original languageEnglish (US)
Pages (from-to)389-395
Number of pages7
JournalJournal of Clinical Investigation
Volume66
Issue number2
StatePublished - 1980
Externally publishedYes

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Mononuclear Leukocytes
Adenosine Deaminase
Leukocytes
Transplants
Erythrocyte Transfusion
Antibody Formation
Immunization
Erythrocytes
Lymphocytes
Leukapheresis
Secondary Immunization
Herpes Simplex
Diphtheria
Homologous Transplantation
Lymphocyte Count
Hemagglutinins
Phytohemagglutinins
Skin Tests
Candida albicans
Fathers

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Immunoreconstitution by peripheral blood leukocytes in adenosine deaminase-deficient severe combined immunodeficiency. / Rich, K. C.; Richman, Carol M; Mejias, E.; Daddona, P.

In: Journal of Clinical Investigation, Vol. 66, No. 2, 1980, p. 389-395.

Research output: Contribution to journalArticle

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