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Polyclonal expansion of CD3 + /CD4 + /CD56 + large granular lymphocytes and autoimmunity associated with dysregulation of Fas/FasL apoptotic pathway
Author(s) -
Camagna Antonio,
Cedrone Letizia,
Caré Alessandra,
Samoggia Paola,
De Marco Maria Civita,
Del Duca Pietro,
De Martinis Carlo,
Testa Ugo
Publication year - 2001
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.2001.02483.x
Subject(s) - fas ligand , fas receptor , autoimmunity , apoptosis , immunology , immune dysregulation , cd3 , polyclonal antibodies , immune system , biology , cancer research , antibody , programmed cell death , cd8 , genetics
Evidence is accumulating regarding CD95/CD95 ligand (Fas/FasL) pathway dysregulation in clonal diseases of the lymphohaemopoietic lineages. According to these observations, it has been proposed that this defect may represent one of the mechanisms of tumour progression. In large granular lymphocyte (LGL) leukaemia, dysregulated apoptosis may represent a key event in the development of malignancy and autoimmunity. This case report describes dysregulation of the Fas/FasL pathway in a chronic polyclonal expansion of CD3 + LGLs associated with numerous serological immune abnormalities.