Abstract：
:In common variable immunodeficiency (CVID) defects in early stages of B-cell development, bone marrow (BM) plasma cells and T lymphocytes have not been studied systematically. Here we report the first morphologic and flow cytometric study of B- and T-cell populations in CVID BM biopsies and aspirates. Whereas the hematopoietic compartment showed no major lineage abnormalities, analysis of the lymphoid compartment exhibited major pathologic alterations. In 94% of the patients, BM plasma cells were either absent or significantly reduced and correlated with serum immunoglobulin G levels. Biopsies from CVID patients had significantly more diffuse and nodular CD3(+) T lymphocyte infiltrates than biopsies from controls. These infiltrates correlated with autoimmune cytopenia but not with other clinical symptoms or with disease duration and peripheral B-cell counts. Nodular T-cell infiltrates correlated significantly with circulating CD4(+)CD45R0(+) memory T cells, elevated soluble IL2-receptor and neopterin serum levels indicating an activated T-cell compartment in most patients. Nine of 25 patients had a partial block in B-cell development at the pre-B-I to pre-B-II stage. Because the developmental block correlates with lower transitional and mature B-cell counts in the periphery, we propose that these patients might form a new subgroup of CVID patients.
journal_name
Bloodjournal_title
Bloodauthors
Ochtrop ML,Goldacker S,May AM,Rizzi M,Draeger R,Hauschke D,Stehfest C,Warnatz K,Goebel H,Technau-Ihling K,Werner M,Salzer U,Eibel H,Schlesier M,Peter HHdoi
10.1182/blood-2010-11-321695subject
Has Abstractpub_date
2011-07-14 00:00:00pages
309-18issue
2eissn
0006-4971issn
1528-0020pii
blood-2010-11-321695journal_volume
118pub_type
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