Abnormal in vitro thymocyte differentiation in a patient with severe combined immunodeficiency-Nezelof's syndrome

An in vitro coculture model system of CD34+ stem cells and allogenic cultured thymic epithelia fragments was used to evaluate thymocyte differentiation in a 9-month-old child of Amish descent with Nezelof syndrome. Though the patient's stem cells differentiate to acquire normal expression of CD...

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Bibliographic Details
Published in:Journal of clinical immunology 1996-05, Vol.16 (3), p.151-158
Main Authors: KNUTSEN, A. P, WALL, D, MUELLER, K. R, BOUHASIN, J. D
Format: Article
Language:English
Subjects:
Antigens, CD34 - biosynthesis
Biological and medical sciences
Cell Differentiation - immunology
Coculture Techniques
Flow Cytometry
Hematopoietic Stem Cells - immunology
Humans
Immunodeficiencies
Immunodeficiencies. Immunoglobulinopathies
Immunopathology
Infant
Male
Medical sciences
Severe Combined Immunodeficiency - immunology
Severe Combined Immunodeficiency - pathology
T-Lymphocytes - pathology
Thymus Gland - immunology
Thymus Gland - pathology
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Summary:An in vitro coculture model system of CD34+ stem cells and allogenic cultured thymic epithelia fragments was used to evaluate thymocyte differentiation in a 9-month-old child of Amish descent with Nezelof syndrome. Though the patient's stem cells differentiate to acquire normal expression of CD2 and CD7, later steps of maturation were abnormal. There was detectable but reduced expression of CD3 and CD4 phenotypes. CD44+ expression, however, was markedly reduced. CD44 is an adhesion molecule, interacting with the matrix ligands hyaluronan and fibronectin, and is expressed early in thymocyte differentiation and subsequently in mature T cells. It is hypothesized that abnormal expression of CD44 in a variant of severe combined immunodeficiency, Nezelof's syndrome, interferes with normal thymocyte and thymic epithelial interaction, which leads to abnormal thymocyte differentiation.
ISSN:0271-9142
1573-2592
DOI:10.1007/BF01540913