TY - JOUR
T1 - Immunological and genotypic analysis of human Tγ-lymphoproliferative disorders
AU - Rambaldi, Alessandro
AU - Allavena, Paola
AU - Pirelli, Anna
AU - Di Bello, Maria
AU - Rossini, Silvano
AU - Bassan, Renato
AU - Barbui, Tiziano
AU - Pelicci, Pier Giuseppe
AU - Favera, Riccardo Dalla
AU - Mantovani, Alberto
PY - 1986/1
Y1 - 1986/1
N2 - Tγ-lymphoproliferative disorders (Tγ-LPD) are rare diseases characterized by expansion of circulating elements with resemblance to large granular lymphocytes (LGL). We have studied 12 patients with Tγ-LPD. Morphological evaluation revealed 79.88% of LGL in non-adherent peripheral blood lymphocytes as assessed by light and electron microscopy. The most common features of the membrane phenotype included expression of T3, HNK-1 and AB8.28 (anti-Fcγ); other surface markers of LGL (OKM1, B73.1, N901) were variably expressed or absent. Patients' LGL usually had little or no NK activity, with the exception of two patients who had values comparable to those of normal donors; in addition, cell preparations from all patients mediated antibody-dependent cellular cytotoxicity. The recent availability of the T cell receptor β chain probes allowed us to investigate the lineage and the clonality of Tγ-LPD. Of the 12 patients analyzed, 10 displayed clonal rearrangements of Tβ locus and expression of the T3 antigen, whereas the two remaining cases displayed a germ-line configuration of the Tβ gene and no expression of the T3 antigen. We suggest that individual Tγ-LPD cases represent the clonal expansion of cells frozen at different stages of differentiation/activation within an individual hematopoietic LGL/NK lineage. These data suggest that either a subset of LGL or a particular step of differentiation may be related to the T cell lineage.
AB - Tγ-lymphoproliferative disorders (Tγ-LPD) are rare diseases characterized by expansion of circulating elements with resemblance to large granular lymphocytes (LGL). We have studied 12 patients with Tγ-LPD. Morphological evaluation revealed 79.88% of LGL in non-adherent peripheral blood lymphocytes as assessed by light and electron microscopy. The most common features of the membrane phenotype included expression of T3, HNK-1 and AB8.28 (anti-Fcγ); other surface markers of LGL (OKM1, B73.1, N901) were variably expressed or absent. Patients' LGL usually had little or no NK activity, with the exception of two patients who had values comparable to those of normal donors; in addition, cell preparations from all patients mediated antibody-dependent cellular cytotoxicity. The recent availability of the T cell receptor β chain probes allowed us to investigate the lineage and the clonality of Tγ-LPD. Of the 12 patients analyzed, 10 displayed clonal rearrangements of Tβ locus and expression of the T3 antigen, whereas the two remaining cases displayed a germ-line configuration of the Tβ gene and no expression of the T3 antigen. We suggest that individual Tγ-LPD cases represent the clonal expansion of cells frozen at different stages of differentiation/activation within an individual hematopoietic LGL/NK lineage. These data suggest that either a subset of LGL or a particular step of differentiation may be related to the T cell lineage.
KW - Antibody-dependent cellular cytotoxicity
KW - LGL leukemia
KW - LGL phenotypic markers
KW - LGL Tγ-lymphoproliferative disease
KW - Lymphokine-activated killer cells
KW - Natural killer activity
KW - T cell receptor β-chain rearrangement
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U2 - 10.1007/BF02886721
DO - 10.1007/BF02886721
M3 - Article
C2 - 3488575
AN - SCOPUS:0022438333
VL - 16
SP - 29
EP - 35
JO - Ricerca in Clinica e in Laboratorio
JF - Ricerca in Clinica e in Laboratorio
SN - 0940-5437
IS - 1
ER -