Abstract
Larges granulars lymphocytes (LGL) leukemias correspond to rare haemopathies secondary to clonal proliferation of larges lymphocytes rich in azurophilic granules with phenotype T of CD3+ or CD3- (NK cell) profile. We report the case of a patient, 75 years old, who was admitted for pancytopenia associated with lymphocytosis at 8G / L all evolving in a febrile context. The clinical examination did not find tumoral syndrome. The blood smear founded very numerous granulars lymphocytes representing 46% of all lymphocytes. The myelogram shows an aspect in favor of peripheral thrombocytopenia without other notable abnormalities. There is a cellular expansion of CD8+ NK T cells compatible with LGL-type proliferation. The bone marrow is infiltrated by these lymphocytes with a percentage of 28%. It is a pathology with an heterogeneous spectrum classified by the World Health Organization into three entities: LGL T leukemia and chronic NK cell expansions with chronic and indolent evolution and aggressive cell leukemia NK with usually a reserved prognosis. Recent advances in the knowledge of the pathophysiology of these haemopathies have made it possible to specify the mechanisms underlying the perpetuation of the LGL clone and to identify new therapeutic targets.
Highlights
Leukemias with large granular lymphocytes (LGL) correspond to rare haemopathies [1], secondary to clonal proliferation of large lymphocytes rich in azurophilic granules of phenotype T of CD3+ or CD3- (NK cell) profile
The characteristics that unite these three heterogeneous entities are based on the clonal expansion of an LGL population greater than 0.4 G/L in peripheral blood, with a characteristic T or NK phenotype over a period of less than six months [2]
LGL T CD3+ leukemias account for 85% of LGL leukemias, whereas NK-cell chronic lymphoproliferative disorders account for only 5% [3]
Summary
Leukemias with large granular lymphocytes (LGL) correspond to rare haemopathies [1], secondary to clonal proliferation of large lymphocytes rich in azurophilic granules of phenotype T of CD3+ or CD3- (NK cell) profile. These lymphocytes constitute 10 to 15% of the total circulating lymphocytes. The characteristics that unite these three heterogeneous entities are based on the clonal expansion of an LGL population greater than 0.4 G/L in peripheral blood, with a characteristic T or NK phenotype over a period of less than six months [2] These criteria make it possible to distinguish LGL leukemias from reactive LGL expansions. Maryam Mouamin et al.: Large Granular Lymphocytes Leukemia: A Case Report with a Review of the Literature
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