Immunodeficiency due to a unique protracted developmental delay in the B-cell lineage

Armond S. Goldman, Stephen E. Miles, Helen E. Rudloff, Kimberly H. Palkowetz, Frank C. Schmalstieg

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

A unique immune deficiency in a 24-month-old male characterized by a transient but protracted developmental delay in the B-cell lineage is reported. Significant deficiencies in the number of B cells in the blood, the concentrations of immunoglobulins in the serum, and the titers of antibodies to T-dependent and T-independent antigens resolved spontaneously by the age of 39 months in a sequence that duplicated the normal development of the B- cell lineage: blood B cells followed by immunoglobulin M (IgM), IgG, IgA, and specific IgG antibodies to T-independent antigens (pneumococcal polysaccharides). Because of the sequence of recovery, the disorder could have been confused with other defects in humoral immunity, depending on when in the course of disease immunologic studies were conducted. Investigations of X-chromosome polymorphisms suggested that the disorder was not X linked in that the mother appeared to have identical X chromosomes. An autosomal recessive disorder involving a gene that controls B-cell development and maturation seems more likely. In summary, this case appears to be a novel protracted delay in the development of the B-cell lineage, possibly due to an autosomal recessive genetic defect.

Original languageEnglish (US)
Pages (from-to)161-167
Number of pages7
JournalClinical and Diagnostic Laboratory Immunology
Volume6
Issue number2
StatePublished - Mar 1999

Fingerprint

Cell Lineage
B-Lymphocytes
Cells
T Independent Antigens
X Chromosome
Chromosomes
Blood
Immunoglobulin G
Defects
Antibodies
Immune System Diseases
Humoral Immunity
Polymorphism
Immunoglobulin A
Polysaccharides
Immunoglobulin M
Immunoglobulins
Blood Cells
Genes
Recovery

ASJC Scopus subject areas

  • Microbiology (medical)
  • Immunology and Allergy
  • Clinical Biochemistry
  • Immunology

Cite this

Goldman, A. S., Miles, S. E., Rudloff, H. E., Palkowetz, K. H., & Schmalstieg, F. C. (1999). Immunodeficiency due to a unique protracted developmental delay in the B-cell lineage. Clinical and Diagnostic Laboratory Immunology, 6(2), 161-167.

Immunodeficiency due to a unique protracted developmental delay in the B-cell lineage. / Goldman, Armond S.; Miles, Stephen E.; Rudloff, Helen E.; Palkowetz, Kimberly H.; Schmalstieg, Frank C.

In: Clinical and Diagnostic Laboratory Immunology, Vol. 6, No. 2, 03.1999, p. 161-167.

Research output: Contribution to journalArticle

Goldman, AS, Miles, SE, Rudloff, HE, Palkowetz, KH & Schmalstieg, FC 1999, 'Immunodeficiency due to a unique protracted developmental delay in the B-cell lineage', Clinical and Diagnostic Laboratory Immunology, vol. 6, no. 2, pp. 161-167.
Goldman AS, Miles SE, Rudloff HE, Palkowetz KH, Schmalstieg FC. Immunodeficiency due to a unique protracted developmental delay in the B-cell lineage. Clinical and Diagnostic Laboratory Immunology. 1999 Mar;6(2):161-167.
Goldman, Armond S. ; Miles, Stephen E. ; Rudloff, Helen E. ; Palkowetz, Kimberly H. ; Schmalstieg, Frank C. / Immunodeficiency due to a unique protracted developmental delay in the B-cell lineage. In: Clinical and Diagnostic Laboratory Immunology. 1999 ; Vol. 6, No. 2. pp. 161-167.
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