J Pediatr. 2006 Mar;148(3):404-6.

A novel form of non-X-linked hyperigm associated with growth and pubertal disturbances and with lymphoma development.

Moschese V, Lintzman J, Callea F, Chini L, Devito R, Carsetti R, Di Cesare S, Geissman F, Brousse N, Rossi P, Durandy A.

Source

Department of Pediatrics, University of Rome Tor Vergata, Italy. moschese@med.uniroma2.it

Abstract

HyperIgM syndrome is a heterogenous immunodeficiency characterized by impaired class-switch recombination due to different molecular abnormalities. We report on two female patients affected by a novel syndrome associating HIGM, growth and pubertal disturbances, and severe lymphoid hyperplasia with eventual development into lymphomas, suggesting a DNA repair defect.

PMID:: 16615979; [PubMed - indexed for MEDLINE]

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Immunoglobulin M

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