Immunological and genotypic analysis of human Tγ-lymphoproliferative disorders

Alessandro Rambaldi, Paola Allavena, Anna Pirelli, Maria Di Bello, Silvano Rossini, Renato Bassan, Tiziano Barbui, Pier Giuseppe Pelicci, Riccardo Dalla Favera, Alberto Mantovani

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)29-35
Number of pages7
JournalLa Ricerca in Clinica e in Laboratorio
Volume16
Issue number1
DOIs
Publication statusPublished - Jan 1986

Keywords

  • Antibody-dependent cellular cytotoxicity
  • LGL leukemia
  • LGL phenotypic markers
  • LGL Tγ-lymphoproliferative disease
  • Lymphokine-activated killer cells
  • Natural killer activity
  • T cell receptor β-chain rearrangement

ASJC Scopus subject areas

  • Clinical Biochemistry

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