Defective anti-polysaccharide response and splenic marginal zone disorganization in ALPS patients.

Abstract:

:Autoimmune lymphoproliferative syndrome (ALPS) caused by impaired FAS-mediated apoptosis of lymphocytes is characterized by lymphoproliferation, autoimmunity, but also an increased risk of invasive bacterial infection, notably following splenectomy. We surveyed a cohort of 100 ALPS patients (including 33 splenectomized) and found that 12 (10 splenectomized) had experienced 23 invasive bacterial infections mainly caused by Streptococcus pneumoniae. This vulnerability was associated with evidence of defective B-cell function characterized by low serum immunoglobulin (Ig) M, low IgM antibody production in response to S pneumoniae following nonconjugated immunization, and low blood memory B-cells counts (including marginal zone [MZ] B-cell counts). This immunodeficiency strongly correlated with intensity of lymphoproliferation. Spleen sections from 9 ALPS patients revealed double-negative T-cell (DN-T) infiltration of the MZ, which was depleted of B cells. MZ in ALPS patients contained an abnormally thick layer of MAdCAM-1((+)) stromal cells and an excess of DN-Ts. DN-Ts were shown to express MAdCAM-1 ligand, the α4β7 integrin. These observations suggest that accumulating DN-Ts are trapped within stromal cell meshwork and interfere with correct localization of MZ B cells. Similar observations were made in spleens of fas-deficient mice. Our data revealed an unexpected mechanism by which ALPS results in anti-polysaccharide IgM antibody production-specific defect. Splenectomy should be avoided.

journal_name

Blood

journal_title

Blood

authors

Neven B,Bruneau J,Stolzenberg MC,Meyts I,Magerus-Chatinet A,Moens L,Lanzarotti N,Weller S,Amiranoff D,Florkin B,Bader-Meunier B,Leverger G,Ferster A,Chantrain C,Blanche S,Picard C,Molina TJ,Brousse N,Durandy A,Rizzi

doi

10.1182/blood-2014-02-553834

subject

Has Abstract

pub_date

2014-09-04 00:00:00

pages

1597-609

issue

10

eissn

0006-4971

issn

1528-0020

pii

blood-2014-02-553834

journal_volume

124

pub_type

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