000			01810cam a2200205 4500500
005			20250112035847.0
041			_afre
042			_adc
100	1	0	_aDrillet, Gaëlle _eauthor
700	1	0	_a Pastoret, Cédric _eauthor
700	1	0	_a Moignet, Aline _eauthor
700	1	0	_a Lamy de la Chapelle, Thierry _eauthor
700	1	0	_a Marchand, Tony _eauthor
245	0	0	_aNK subtype of large granular lymphocytic leukemia
260			_c2022.
500			_a38
520			_aLarge granular lymphocytic leukemias (LGLs) are rare lymphoproliferative syndromes characterized by the clonal expansion of T or NK lymphocytes in 85 and 15% of cases respectively. Interestingly, T- and NK- LGL leukemias share a common pathophysiology and similar clinical and biological presentations. This lymphoproliferative syndrome is characterized by cytopenias and a frequent association with autoimmune diseases or manifestations. It is an indolent disease that in most cases allows for an abstinence-only strategy at diagnosis. However, the majority of patients will require initiation of treatment during follow-up. As NK cells lack a TCR, obtaining evidence of clonality in NK-LGL leukemias is difficult. This is crucial in view of possible reactive expansions in the context of viral infections or dysimmune diseases. The diagnostic approach has been facilitated by the progress made in recent years in the understanding of the pathophysiology and the recent identification of recurrent mutations. In this review, we will discuss the pathophysiology of NK-LGL leukemias and present recent advances in diagnostic strategies before discussing therapeutic management.
786	0		_nHématologie | 28 | 5 | 2022-09-01 | p. 232-245 | 1264-7527
856	4	1	_uhttps://shs.cairn.info/journal-hematologie-2022-5-page-232?lang=en
999			_c174293 _d174293