title: CD3+CD4-CD8+NK- large granular lymphocytosis with neutropenia and evidence for clonality and T-cell receptor gene rearrangement: two pediatric cases.
abstract: The authors describe two pediatric cases of large granular lymphocytosis presenting early in the second decade of life with neutropenia and sepsis. They are among the youngest described in the literature. This report focuses on the advantages of detailed immunophenotypic and molecular analysis and highlights some of the controversies and uncertainties in the management of these patients, particularly the choice of immunosuppressive therapy. Immunosuppressive therapy in the two children described in this report resulted in improvement of neutropenia and clinical status, but this was not accompanied by the disappearance of the clonal population.
authors.affiliation: Department of Immunology, Southampton General Hospital, Southampton, UK. mtkrishna@yahoo.com
authors.forename: Mamidipudi T
authors.initials: MT
authors.lastname: Krishna
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Hodges
authors.forename: Frances L
authors.initials: FL
authors.lastname: Lavender
authors.forename: Susan
authors.initials: S
authors.lastname: Harris
authors.forename: Andrew
authors.initials: A
authors.lastname: Gennery
authors.forename: Andrew
authors.initials: A
authors.lastname: Cant
authors.forename: Brenda
authors.initials: B
authors.lastname: Gibson
authors.forename: Rosalie
authors.initials: R
authors.lastname: Wilkie
authors.forename: Philip
authors.initials: P
authors.lastname: Darbyshire
authors.forename: John L
authors.initials: JL
authors.lastname: Smith
mesh_terms: D017252:CD3 Complex / Q000276:immunology*; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology*; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology*; D002648:Child; D002999:Clone Cells; D003131:Combined Modality Therapy; D016572:Cyclosporine / Q000627:therapeutic use; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor / Q000235:genetics*; D019674:Genes, T-Cell Receptor beta / Q000235:genetics; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006801:Humans; D016130:Immunophenotyping; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007694:Killer Cells, Natural / Q000276:immunology*; D007958:Leukocyte Count; D008218:Lymphocytosis / Q000150:complications / Q000276:immunology* / Q000628:therapy; D008297:Male; D009503:Neutropenia / Q000150:complications / Q000276:immunology* / Q000628:therapy; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000235:genetics / Q000276:immunology*
chemical_list: D017252:CD3 Complex; D007166:Immunosuppressive Agents; D016693:Receptors, Antigen, T-Cell, alpha-beta; D016179:Granulocyte Colony-Stimulating Factor; D016572:Cyclosporine