Association of clonal T-cell large granular lymphocyte disease and paroxysmal nocturnal haemoglobinuria (PNH): further evidence for a pathogenetic link between T cells, aplastic anaemia and PNH

Autor:	A, Karadimitris, K, Li, R, Notaro, D J, Araten, K, Nafa, R, Thertulien, M, Ladanyi, A E, Stevens, C S, Rosenfeld, I A, Roberts, L, Luzzatto
Rok vydání:	2002
Předmět:	Adult Leukemia T-Cell T-Lymphocytes Hemoglobinuria Paroxysmal Anemia Aplastic Humans Female Gene Rearrangement beta-Chain T-Cell Antigen Receptor Hematopoietic Stem Cells Polymerase Chain Reaction Cell Division Coculture Techniques Clone Cells
Zdroj:	British journal of haematology. 115(4)
ISSN:	0007-1048
Popis:	There is mounting evidence to suggest that T-cell-mediated suppression of haemopoiesis is a pathogenetic mechanism in three bone marrow failure syndromes: aplastic anaemia (AA), paroxysmal nocturnal haemoglobinuria (PNH) and myelodysplasia (MDS). T-cell microclones can be detected by sensitive polymerase chain reaction (PCR)-based methods in all three disorders. Recently, larger clonal populations of T-cell large granular lymphocytes (T-LGLs) have been observed in some patients with AA and MDS. Here, we report the development of a large clonal T-LGL population in a patient with bona fide PNH. In this patient, we defined part of the sequence of the T-cell receptor (TCR) beta-chain gene, and we have shown that the large T-LGL population emerged from a background of multiple smaller T-cell clones. Thus, T-LGL clones in AA, MDS and PNH probably expand as a result of antigenic stimulation. It is postulated that the antigen driving clonal T-cell proliferations in these disorders exists on haemopoietic stem cells.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=pmid________::dce8c16e6c22913ba801d01fe58a582c https://pubmed.ncbi.nlm.nih.gov/11843843 Zobrazit plný text záznamu Plný text