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Clinical and Diagnostic Laboratory Immunology, March 1999, p. 161-167, Vol. 6, No. 2
1071-412X/99/$04.00+0
Copyright © 1999, American Society for Microbiology. All rights reserved.

Immunodeficiency Due to a Unique Protracted Developmental Delay in the B-Cell Lineage

Armond S. Goldman,1,2,3,4,* Stephen E. Miles,1 Helen E. Rudloff,1 Kimberly H. Palkowetz,1 and Frank C. Schmalstieg Jr.1

Department of Pediatrics,1 Microbiology and Immunology,2 Pathology,3 and Human Biological Chemistry and Genetics4 of the University of Texas Medical Branch, Galveston, Texas

Received 15 July 1998/Returned for modification 27 August 1998/Accepted 7 October 1998

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.


* Corresponding author. Mailing address: Pediatric Immunology/Allergy/Rheumatology Division, Room 2.360 Children's Hospital, The University of Texas Medical Branch, 301 University Blvd., Galveston, TX 77555-0369. Phone: (409) 772-2658. Fax: (409) 747-6622. E-mail: agoldman{at}utmb.edu.


Clinical and Diagnostic Laboratory Immunology, March 1999, p. 161-167, Vol. 6, No. 2
1071-412X/99/$04.00+0
Copyright © 1999, American Society for Microbiology. All rights reserved.






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