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.
journal_name
Cancerjournal_title
Cancerauthors
Le Deist F,de Saint Basile G,Coulombel L,Breton-Gorius J,Maier-Redelsperger M,Beljorde K,Bremard C,Griscelli Cdoi
10.1002/1097-0142(19910515)67:10<2610::aid-cncr282subject
Has Abstractpub_date
1991-05-15 00:00:00pages
2610-7issue
10eissn
0008-543Xissn
1097-0142journal_volume
67pub_type
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