Large granular lymphocyte leukemia (LGL L) has been morphologically characterized as a group of lymphoproliferative diseases that include T-cell large granular lymphocytic leukemia (T-LGL L) and chronic lymphoproliferative disorders of natural killer cells (CLPD-NK). We investigated mutations in the Src homology 2 (SH2) domain of the signal transducer and activator of transcription 3 (STAT3) ge...

Large granular lymphocyte (LGL) leukemia is an uncommon lymphoproliferative disorder characterized by an expansion of CD3' and less frequently CD3lymphocytes.' In more than 80% of cases, severe neutropenia (neutrophils <0.5 X 109/L) is observed? The mechanism responsible for inhibition of granulopoiesis remains controversial. LGL proliferation may suppress granulopoiesis either directly or indi...

A high frequency of CD4(+) T-cell large granular lymphocyte (T-LGL) lymphocytosis occurs in human leukocyte antigen (HLA) -DRB1*0701 individuals displaying monoclonal expansions of Vβ13.1+ CD4(+) T-cell clones, which specifically respond to human cytomegalovirus (HCMV) antigens. We previously reported the expression of natural killer (NK)-cell associated receptors (NKR) by HCMV-specific cytolyt...

BACKGROUND T-cell large granular lymphocytic (T-LGL) leukemia is a rare, chronic, often indolent lymphoproliferative disorder of mature T cells (CD3+). Severe neutropenia and other cytopenias are common features in patients with T-LGL leukemia and may cause infections, thus representing a major cause of morbidity in this disease. Immunosuppressive therapy with low-dose regimes of methotrexate, ...

Large granular lymphocyte (LGL) leukemia is a rare disease characterized by clonal expansion of LGL associated with chronic neutropenia, multiple auto-antibodies, and occasionally polyarthritis. We studied cell surface antigen expression and functional activity of leukemic LGL from ten such patients. Using two-color flow cytometric analysis, we found that leukemic LGL from all ten patients expr...

Large granular lymphocyte (LGL) leukemia represents a spectrum of rare lymphoproliferative diseases defined by clonal amplification of either CD3(+) cytotoxic T-lymphocytes or CD3(-) natural killer cells. This chapter focuses on the T-cell form of LGL leukemia. Clinical features include neutropenia, anemia, and rheumatoid arthritis. LGL leukemia is thought to arise from chronic antigenic stimul...

Biosynthetic labeling with [35S]met and immunoprecipitation with anti-C-reactive protein (CRP) antibodies and Staphylococcus aureus indicate that cell surface CRP is produced by lymphocytes. The ability of anti-CRP to reduce NK activity, and the demonstration that 125I-anti-CRP-labeled PBL are found in low-density Percoll fractions associated with large granular lymphocyte (LGL) and NK activity...

A 9-year-old neutered male Munchkin cat presented to our clinic with lethargy and anorexia. Abdominal ultrasonography revealed multiple enlarged mesenteric lymph nodes. Cytology immunocytochemical staining suggested large granular lymphocyte (LGL) lymphoma derived from cytotoxic T cells. Treatment nimustine (ACNU) (25 mg/m2, intravenously, every 3 weeks) resulted in long-term complete remission...

Morbidity and mortality in patients with T large granular lymphocyte (T-LGL) leukemia resutt from infections acquired during severe neutropenia. Optimum treatment for severe neutropenia remains undefined. We conducted an uncontrolled but prospective study of low-dose oral methotrexate, up to 10 mg/ma weekly, in 10 patients with this disease. Therapeutic response was assessed by serial clinical ...