A lymphoproliferative disorder of the large granular lymphocytes with natural killer activity

Manlio Ferrarini, Sergio Romagnani, Elisabetta Montesoro, Antonio Zicca, Gian Franco del Prete, Arcangelo Nocera, Enrico Maggi, Arnaldo Leprini, Carlo Enrico Grossi

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Abstract

This paper reports the case of a patient with an abnormally expanded population of circulating lymphoid cells displaying the features of the so-called large granular lymphocytes (LGL). These cells were peroxidase negative and nonphagocytic, formed rosettes with sheep erythrocytes, had receptors for IgG, and contained azurophilic (electron-dense) granules. Like normal LGL, the patient cells were positive for two acid hydrolases (acid phosphatase and β-glucuronidase) but did not stain for α-naphthyl acetate esterase (ANAE), which is present in normal LGL. Ultrastructural studies revealed that the patient cells were rich in Golgi-derived vesicles, coated vesicles, multivesicular bodies, and immature granules, indicating that, unlike normal LGL, they were engaged in granulogenesis. These features, together with the absence of ANAE activity, are suggestive of some degree of cell immaturity. The patient cells displayed natural killer (NK) and antibody-dependent cellular cytotoxicity (ADCC) activities comparable to those of normal peripheral blood mononuclear cells, or even higher, and did not respond to T-cell mitogens or allogeneic cells. Furthermore, they were incapable of suppressing normal T-cell proliferation or pokeweed mitogen-induced B-cell differentiation. Analysis of the NK activity at the single-cell level revealed that a large proportion of the patient cells bound to the K562 target cells but could not accomplish the entire lytic process. This finding supports further the possibility that the patient cells were immature LGL. The surface phenotype of the patient cells (as defined by a battery of monoclonal antibodies) was somewhat different from that usually observed in the majority of the normal LGL because, in addition to the HNK-1 marker, the cells were OKT3+, aLeul+, aLeu4+, OKT8+, aLeu2a+, and 3A1+ but were OKM1- and 4F2-. This phenotype could correspond to that of maturing LGL.

Original languageEnglish
Pages (from-to)30-41
Number of pages12
JournalJournal of Clinical Immunology
Volume3
Issue number1
DOIs
Publication statusPublished - Jan 1983

Fingerprint

Lymphoproliferative Disorders
Lymphocytes
Naphthol AS D Esterase
Acetylesterase
Coated Vesicles
Multivesicular Bodies
T-Lymphocytes
Phenotype
IgG Receptors
Muromonab-CD3
Pokeweed Mitogens
K562 Cells
Glucuronidase
Hydrolases
Acid Phosphatase
Mitogens
Natural Killer Cells
Peroxidase
Cell Differentiation
Blood Cells

Keywords

  • large granular lymphocytes
  • Natural killer cells

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

Ferrarini, M., Romagnani, S., Montesoro, E., Zicca, A., del Prete, G. F., Nocera, A., ... Grossi, C. E. (1983). A lymphoproliferative disorder of the large granular lymphocytes with natural killer activity. Journal of Clinical Immunology, 3(1), 30-41. https://doi.org/10.1007/BF00919136

A lymphoproliferative disorder of the large granular lymphocytes with natural killer activity. / Ferrarini, Manlio; Romagnani, Sergio; Montesoro, Elisabetta; Zicca, Antonio; del Prete, Gian Franco; Nocera, Arcangelo; Maggi, Enrico; Leprini, Arnaldo; Grossi, Carlo Enrico.

In: Journal of Clinical Immunology, Vol. 3, No. 1, 01.1983, p. 30-41.

Research output: Contribution to journalArticle

Ferrarini, M, Romagnani, S, Montesoro, E, Zicca, A, del Prete, GF, Nocera, A, Maggi, E, Leprini, A & Grossi, CE 1983, 'A lymphoproliferative disorder of the large granular lymphocytes with natural killer activity', Journal of Clinical Immunology, vol. 3, no. 1, pp. 30-41. https://doi.org/10.1007/BF00919136
Ferrarini, Manlio ; Romagnani, Sergio ; Montesoro, Elisabetta ; Zicca, Antonio ; del Prete, Gian Franco ; Nocera, Arcangelo ; Maggi, Enrico ; Leprini, Arnaldo ; Grossi, Carlo Enrico. / A lymphoproliferative disorder of the large granular lymphocytes with natural killer activity. In: Journal of Clinical Immunology. 1983 ; Vol. 3, No. 1. pp. 30-41.
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