A biclonal large granular lymphocyte (LGL)/NK-associated (NKa) disorder of CD4+ and CD8+ lymphocyte subpopulations characterized by the simultaneous presence of distinct TCR rearrangements.
This communication reports a patient with concomitant expansions of CD4+ and CD8+ large granular lymphocytes. Immunological analyses revealed that the abnormally increased CD4+ LGL fraction was phenotypically similar to other reported persistent CD4+ LGL expansions, whereas the phenotypic profile for the CD8+ LGL component was unusual. Of particular note was the finding that both the CD4+ and CD8+ LGL fractions showed high membrane CD45RO isoform expression, thus suggesting their 'primed' status. Molecular biology studies of immunomagnetically fractionated cells using a T gamma 9 TCR gamma gene primer further revealed that the CD4+ and CD8+ components were both clonal but showed different patterns of rearrangement. It is suggested that the simultaneous presence of CD4+ and CD8+ clonal populations are unlikely to have been derived from a common progenitor and that they reflect expansions of functionally restricted subpopulations.
Richards
S J
SJ
Haematological Malignancy Diagnostic Service, Leeds General Infirmary.
Short
M
M
Steed
A J
AJ
Scott
C S
CS
D000368:Aged; D001483:Base Sequence; D015496:CD4-Positive T-Lymphocytes / Q000473:pathology*; D002999:Clone Cells; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D006098:Granulocytes / Q000473:pathology*; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology; D017493:Leukocyte Common Antigens; D008232:Lymphoproliferative Disorders / Q000235:genetics / Q000473:pathology*; D008969:Molecular Sequence Data; D016176:T-Lymphocyte Subsets / Q000473:pathology*
D017493:Leukocyte Common Antigens