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Ferrata Storti Foundation, Haematologica, 12(99), p. 1826-1833

DOI: 10.3324/haematol.2014.105726

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Detection of monoclonal T populations in patients with KIR-restricted chronic lymphoproliferative disorder of NK cells

This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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Abstract

The etiology of chronic large granular lymphocyte proliferations, either T or Natural Killer, is largely unknown. Although these disorders are characterized by the expansion of different cells types (T and Natural Killer) with specific genetic features and abnormalities, several lines of evidence suggest a common pathogenetic mechanism. According to this interpretation, we speculated that in patients with Natural Killer-type Chronic lymphoproliferative Disorder, together with Natural Killer cells, also T lymphocytes should undergo a persistent antigenic pressure, possibly resulting in an ultimate clonal T cell selection. To strengthen this hypothesis, we evaluated whether clonal T cell populations were detectable in 48 patients with Killer Immunoglobulin-like Receptor restricted Natural Killer-type Chronic lymphoproliferative Disorder. At the time of diagnosis, in a half of patients studied, we found a clearly defined clonal T cell population, despite the fact that all cases presented with a well characterized Natural Killer disorder. Follow up analysis confirmed that TCR gamma rearrangements were stable over the time; furthermore, in seven patients we demonstrated the appearance of a clonal T subset which progressively grows up, leading to a switch between Killer Immunoglobulin-like Receptor restricted Natural Killer-type Disorder to a monoclonal T Large Leukemia. Results of this study support the hypothesis that a common mechanism is involved in the pathogenesis of these disorders.