Polyclonal expansion of CD3(+)/CD4(+)/CD56(+) large granular lymphocytes and autoimmunity associated with dysregulation of Fas/FasL apoptotic pathway.
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.
Camagna
A
A
Department of Clinical Sciences, University La Sapienza, Rome, Italy. u.testa@ema.net.iss.it
Cedrone
L
L
Caré
A
A
Samoggia
P
P
De Marco
M C
MC
Del Duca
P
P
De Martinis
C
C
Testa
U
U
D000911:Antibodies, Monoclonal / Q000494:pharmacology; D017209:Apoptosis; D015551:Autoimmunity; D015139:Blotting, Southern; D017252:CD3 Complex; D015704:CD4 Antigens; D019002:CD56 Antigen; D016022:Case-Control Studies; D018450:Disease Progression; D053222:Fas Ligand Protein; D005260:Female; D005434:Flow Cytometry; D018922:HL-60 Cells; D006801:Humans; D007376:Interleukin-2 / Q000494:pharmacology; D015458:Leukemia, T-Cell / Q000276:immunology*; D008213:Lymphocyte Activation; D008562:Membrane Glycoproteins / Q000378:metabolism; D008875:Middle Aged; D013601:T-Lymphocytes / Q000276:immunology*; D019014:fas Receptor / Q000378:metabolism
D000911:Antibodies, Monoclonal; D017252:CD3 Complex; D015704:CD4 Antigens; D019002:CD56 Antigen; C505661:FASLG protein, human; D053222:Fas Ligand Protein; D007376:Interleukin-2; D008562:Membrane Glycoproteins; D019014:fas Receptor