Histopathologic abnormalities of the lymphoreticular tissues in organic cation transporter 2 deficiency: evidence for impaired B cell maturation

Katalin Komlósi; Viktória Havasi; Judit Bene; Norbert Süle; László Pajor; Raffaella Nicolai; Paola Benatti; Menotti Calvani; Béla Melegh

doi:10.1016/j.jpeds.2006.09.042

Histopathologic abnormalities of the lymphoreticular tissues in organic cation transporter 2 deficiency: evidence for impaired B cell maturation

J Pediatr. 2007 Jan;150(1):109-111.e2. doi: 10.1016/j.jpeds.2006.09.042.

Authors

Katalin Komlósi¹, Viktória Havasi, Judit Bene, Norbert Süle, László Pajor, Raffaella Nicolai, Paola Benatti, Menotti Calvani, Béla Melegh

Affiliation

¹ Department of Medical Genetics and Child Development, University of Pécs, Pécs, Hungary.

PMID: 17188629
DOI: 10.1016/j.jpeds.2006.09.042

Abstract

Immunohistology of lymphoreticular tissues of a fatal case of organic cation transporter 2 deficiency revealed inhibited proliferation with increased apoptosis in the germinal centers, resulting in "burned out" follicles. This is indicative of impaired antigen driven B cell affinity maturation. Defective humoral immune response might explain the recurrent infections in untreated organic cation transporter 2 deficiency.

Publication types

Case Reports
Research Support, Non-U.S. Gov't

MeSH terms

B-Lymphocytes / pathology*
Cell Enlargement*
Cell Proliferation
Child, Preschool
Disease Progression
Humans
Infant
Lymphoid Tissue / metabolism
Lymphoid Tissue / pathology*
Male
Organic Cation Transport Proteins / deficiency*
Respiratory Tract Infections / immunology
Respiratory Tract Infections / metabolism
Respiratory Tract Infections / pathology*
Solute Carrier Family 22 Member 5

Substances

Organic Cation Transport Proteins
SLC22A5 protein, human
Solute Carrier Family 22 Member 5