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Clonal diseases of large granular lymphocytes Loughran TP JrBlood 1993[Jul]; 82 (1): 1-14Three distinct clinical syndromes occur in patients with increased numbers of circulating LGL. Patients with T-LGL leukemia have clonal proliferations of CD3+ LGL typically associated with chronic neutropenia and autoimmune features. NK-LGL leukemia is characterized by clonal CD3- LGL proliferation with an acute clinical presentation marked by massive hepatosplenomegaly and systemic illness. However, most patients with increased numbers of CD3- LGL do not have clinical features of NK-LGL leukemia and have a chronic clinical course. X-linked gene analyses have supported a polyclonal LGL lymphocytosis in this syndrome. Further studies are needed to determine whether clonal progression can occur in these patients.|CD3 Complex/analysis[MESH]|Clone Cells[MESH]|Female[MESH]|Hematopoiesis[MESH]|Humans[MESH]|Killer Cells, Natural/*pathology[MESH]|Leukemia, Prolymphocytic, T-Cell/*pathology/therapy[MESH]|Leukocyte Count[MESH]|Lymphocyte Subsets[MESH]|Lymphoproliferative Disorders/*pathology[MESH]|Male[MESH]|Middle Aged[MESH]|Prognosis[MESH]|T-Lymphocytes/*pathology[MESH] |
