TY - JOUR
T1 - Clonal expansion of CD8+ BV8 T lymphocytes in bone marrow characterizes thymoma-associated B lymphopenia
AU - Maria Masci, Anna
AU - Palmieri, Giovannella
AU - Vitiello, Laura
AU - Montella, Liliana
AU - Perna, Francesco
AU - Orlandi, Paola
AU - Abbate, Gianfranco
AU - Zappacosta, Serafino
AU - De Palma, Raffaele
AU - Racioppi, Luigi
PY - 2003/4/15
Y1 - 2003/4/15
N2 - A subgroup of thymoma patients is affected by severe immunodeficiency clinically resembling an HIV infection (Good syndrome). These individuals are characterized by B lymphopenia with B-lymphopoiesis deficiency. To investigate the pathogenesis of this unique condition, we studied the T-cell repertoire in blood and bone marrow samples by heterogeneity length analysis of CDR3 beta variable regions of the T-cell receptor (spectratyping). While no alterations were found in the peripheral blood, we detected an oligoclonal population of β variable 8 (BV8) CD8+ T cells in 5 of 5 bone marrow samples. No lymphocyte expansions were found in the bone marrow of 2 thymoma patients with normal B-cell counts, 2 healthy donors, and 3 patients with diseases unrelated to thymoma. These data suggest that an immune response toward an unknown antigen is taking place in the bone marrow of B-lymphopenic thymoma patients. We propose that BV8 CD8+ T cells may play a role in the pathogenesis of this immunodeficiency syndrome.
AB - A subgroup of thymoma patients is affected by severe immunodeficiency clinically resembling an HIV infection (Good syndrome). These individuals are characterized by B lymphopenia with B-lymphopoiesis deficiency. To investigate the pathogenesis of this unique condition, we studied the T-cell repertoire in blood and bone marrow samples by heterogeneity length analysis of CDR3 beta variable regions of the T-cell receptor (spectratyping). While no alterations were found in the peripheral blood, we detected an oligoclonal population of β variable 8 (BV8) CD8+ T cells in 5 of 5 bone marrow samples. No lymphocyte expansions were found in the bone marrow of 2 thymoma patients with normal B-cell counts, 2 healthy donors, and 3 patients with diseases unrelated to thymoma. These data suggest that an immune response toward an unknown antigen is taking place in the bone marrow of B-lymphopenic thymoma patients. We propose that BV8 CD8+ T cells may play a role in the pathogenesis of this immunodeficiency syndrome.
UR - http://www.scopus.com/inward/record.url?scp=0038717675&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0038717675&partnerID=8YFLogxK
U2 - 10.1182/blood-2002-08-2638
DO - 10.1182/blood-2002-08-2638
M3 - Article
C2 - 12515721
AN - SCOPUS:0038717675
VL - 101
SP - 3106
EP - 3108
JO - Blood
JF - Blood
SN - 0006-4971
IS - 8
ER -