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Mastrodemou S, Stalika E, Vardi A, Gemenetzi K, Spanoudakis M, Karypidou M, Mavroudi I, Hadzidimitriou A, Stavropoulos-Giokas C, Papadaki HA, Stamatopoulos K
Leuk. Lymphoma 58 (12) 2926-2933 [2017-12-00; online 2017-05-23]
Chronic idiopathic neutropenia (CIN) is an acquired disorder of granulopoiesis characterized by female predominance and mostly uncomplicated course. Crucial to CIN pathophysiology is the presence of activated T lymphocytes with myelosuppressive properties in both peripheral blood (PB) and bone marrow (BM). We systematically profiled the T cell receptor beta chain (TRB) gene repertoire in CD8+ cells of 34 CIN patients through subcloning/Sanger sequencing analysis of TRBV-TRBD-TRBJ gene rearrangements. Remarkable repertoire skewing and oligoclonality were observed, along with shared clonotypes between different patients, alluding to antigen selection. Cross-comparison of our sequence dataset with public TRB sequence databases revealed that CIN may rarely share common immunogenetic features with other entities, however, the CIN TRB repertoire is largely disease-biased. Overall, these findings suggest that CIN may be driven by long-term exposure to a restricted set of specific CIN-associated antigens.
PubMed 28535087
DOI 10.1080/10428194.2017.1324154
Crossref 10.1080/10428194.2017.1324154