title: A familial occurrence of natural killer cell--T-lymphocyte proliferation disease in two children.
abstract: Several reports describe the association of hyperlymphocytosis with neutropenia. This syndrome, named lymphoproliferative disease, is characterized by a chronic indolent clinical course, bone marrow lymphocyte infiltration, and granulopenia of central origin. The proliferating lymphocytes share large granular lymphocyte natural killer cell and T-lymphocyte characteristics. They are either of monoclonal or polyclonal origin. In this report the familial occurrence of a similar syndrome observed in two children is described. Lymphocyte morphologic abnormalities including nuclear pockets, were noted, a feature usually present in leukemic cells. Lymphocyte proliferation was distinct in each case as shown by the presence of a predominant CD4+ cell population in one and a predominant CD8+ population in the other. Monoclonal gene rearrangements of T-cell receptor beta-chain gene were found although clonal variations occurred with time in one patient. The cause of this unique familial occurrence of monoclonal lymphoproliferation associated with neutropenia is unknown.
authors.affiliation: Unité d'Immunologie et de Rhumatologie, INSERM U 132, Hôpital Necker-Enfants Malades, Paris, France.
authors.forename: F
authors.initials: F
authors.lastname: Le Deist
authors.forename: G
authors.initials: G
authors.lastname: de Saint Basile
authors.forename: L
authors.initials: L
authors.lastname: Coulombel
authors.forename: J
authors.initials: J
authors.lastname: Breton-Gorius
authors.forename: M
authors.initials: M
authors.lastname: Maier-Redelsperger
authors.forename: K
authors.initials: K
authors.lastname: Beljorde
authors.forename: C
authors.initials: C
authors.lastname: Bremard
authors.forename: C
authors.initials: C
authors.lastname: Griscelli
mesh_terms: D000293:Adolescent; D000380:Agranulocytosis / Q000150:complications*; D000945:Antigens, Differentiation, T-Lymphocyte / Q000032:analysis; D002467:Cell Nucleus / Q000473:pathology; D005190:Family; D005260:Female; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; D006412:Hematopoietic Stem Cells / Q000378:metabolism; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology; D007958:Leukocyte Count; D008232:Lymphoproliferative Disorders / Q000097:blood / Q000209:etiology* / Q000235:genetics; D008297:Male; D010641:Phenotype; D013601:T-Lymphocytes / Q000473:pathology
chemical_list: D000945:Antigens, Differentiation, T-Lymphocyte