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A familial occurrence of natural killer cell‐T‐lymphocyte proliferation disease in two children
Author(s) -
Le Deist Françoise,
De Saint Basile Geneviève,
Bremard Clotilde,
Griscelli Claude,
Coulombel Laure,
BretonGorius Jeanine,
MaierRedelsperger Micheline,
Beljorde Kheira
Publication year - 1991
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19910515)67:10<2610::aid-cncr2820671035>3.0.co;2-w
Subject(s) - lymphocyte , monoclonal , immunology , lymphoproliferative disorders , cd8 , natural killer cell , neutropenia , population , bone marrow , medicine , t lymphocyte , lymphoma , monoclonal antibody , gene rearrangement , biology , pathology , cytotoxic t cell , antibody , gene , genetics , immune system , chemotherapy , environmental health , in vitro
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 β‐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.