T-LGL leukemia

GPTKB entity

Statements (49)
Predicate Object
gptkbp:instanceOf gptkb:disease
leukemia
lymphoproliferative disorder
gptkbp:affects gptkb:T_cells
gptkbp:associatedWith gptkb:anemia
autoimmune disorders
splenomegaly
neutropenia
rheumatoid arthritis
gptkbp:cellType CD3+ T cells
CD8+ cytotoxic T cells
gptkbp:characterizedBy clonal proliferation of large granular lymphocytes
gptkbp:commonMarker gptkb:CD3
gptkb:CD8
gptkb:CD57
CD16
TCR alpha/beta
gptkbp:diagnosedBy peripheral blood smear
flow cytometry
T-cell receptor gene rearrangement studies
gptkbp:differentialDiagnosis myelodysplastic syndrome
chronic lymphocytic leukemia
NK-LGL leukemia
gptkbp:firstDescribed 1985
gptkbp:fullName gptkb:T-cell_large_granular_lymphocytic_leukemia
gptkbp:gender slightly more common in males
https://www.w3.org/2000/01/rdf-schema#label T-LGL leukemia
gptkbp:ICD-10_code gptkb:C91.8
gptkbp:incidence rare
gptkbp:medianAge 60 years
gptkbp:mutationAssociatedWith gptkb:STAT3_mutation
gptkb:STAT5b_mutation
gptkbp:notableFeature can be associated with Felty's syndrome
can be associated with pure red cell aplasia
can be asymptomatic
chronic course
often requires long-term management
gptkbp:prevalence less than 5% of all chronic lymphoproliferative disorders
gptkbp:prognosis generally indolent
gptkbp:symptom fatigue
recurrent infections
cytopenias
gptkbp:treatment gptkb:cyclophosphamide
gptkb:cyclosporine
gptkb:prednisone
gptkb:methotrexate
immunosuppressive therapy
gptkbp:bfsParent gptkb:T-cell_LGL_Leukemia
gptkbp:bfsLayer 8