Pathogenesis and phenotypes of an X-linked recessive lymphoproliferative syndrome.

Lancet (London, England) ·Vol. 2 ·No. 7991 ·1976-10-23 ·Pages 882-5

Purtilo DT

Abstract

A new X-linked recessive lymphoproliferative syndrome has variable phenotypes: fatal infectious mononucleosis (I.M.), agammaglobulinaemia after I.M., American Burkitt's lymphoma, histiocytic lymphoma, immunoblastic sarcoma of B cells, or plasmacytoma. An immunodeficiency to rubeola and the Epstein-Barr virus probably ensues from the mutant gene. The phenotypes (spectrum of B-cell disorders) have a common inheritance and the aetiology is similar.

MeSH Terms

Adolescent Adult Agammaglobulinemia/etiology,genetics B-Lymphocytes/immunology Child Child, Preschool Female Herpesvirus 4, Human/immunology Humans Immunologic Deficiency Syndromes/complications Infant Infectious Mononucleosis/etiology,genetics Lymphoma/etiology,genetics Lymphoma, Large B-Cell, Diffuse/genetics,immunology Male Phenotype Plasmacytoma/etiology,genetics Sarcoma/etiology,genetics Sex Chromosome Aberrations/complications Syndrome

Authors & Affiliations

1 authors, click to expand affiliations / ORCID

Purtilo D T

Article Info

Journal

Lancet (London, England)

Abbr.

Lancet

ISSN

0140-6736

DOI

10.1016/s0140-6736(76)90542-0

Published

1976-10-23

Pages

882-5

Language

English

Region

England

NLM ID

2985213R

Subset

External Links

PubMed source DOI: 10.1016/s0140-6736(76)90542-0 View journal details

Analysis Services

Pathogenesis and phenotypes of an X-linked recessive lymphoproliferative syndrome.

Contact

WeChat Official Account