Parallel tubular arrays in severe combined immunodeficiency disease: an ultrastructural study of peripheral blood lymphocytes

Payne, CM; Jf, Jones; Sieber, OF Jr; Va, Fulginiti

doi:10.1182/blood.v50.1.55.55

Blood

1977

DOI: 10.1182/blood.v50.1.55.55

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Parallel tubular arrays in severe combined immunodeficiency disease: an ultrastructural study of peripheral blood lymphocytes

CM Payne¹,

Jones Jf²,

OF Jr Sieber³

et al.

Abstract: The ultrastructure of the lymphocytes from three children with severe combined immunodeficiency disease (SCID) is described. Parallel tubular arrays (PTA) were found in a large percentage of circulating lymphocytes (53%, 41%, and 13%) in three SCID patients when compared to age-matched controls. The size of these inclusions was quite variable, with some attaining a length of 1.7 micrometer. They contained a tubular substructure with a diameter of 36--44 nm. The PTA were mostly located in the centriolar and Gol… Show more

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1983

1987

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Cited by 18 publications

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A T chronic lymphocytic leukemia with large granular lymphocytes phenotype and functions of leukemic cells under in vitro treatment by differentiation inducers

Merle‐Béral¹,

Boucheix²,

Karray³

et al. 1987

Cancer

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We reported the morphologic, phenotypic and functional characteristics of leukemic cells with natural killer (NK) properties in a case of T chronic lymphocytic leukemia with large granular lymphocytes. These cells were cytologically and cytochemically characterized as phosphatase acid positive large granular lymphocytes (LGL), and presented parallel tubular arrays at the ultra structural level. They displayed a CD2, CD3, CD8, CD11, Leu 7, and Leu 11 positive phenotype while they lacked B cell markers including surface immunoglobulins. In addition, they expressed human leukocyte antigens (HLA) Class I, but no Class II antigens. These phenotypic studies were also performed after cells were cultured in vitro with 12-0-tetradecanoyl phorbol 13-acetate, gamma interferon, 5-azacytidine, sodium butyrate, phytohemagglutinin, and interleukin 2 (IL2). The cell surface markers underwent several significant changes. Among them we noted a higher percentage of labeled cells with anti-CD6 and CD7 monoclonal antibodies (moAbs), and a positivity with an anti-CD19 (B4) moAb. The leukemic LGL spontaneously developed a NK activity on K 562 tumor cells, which was not affected under the various T and B cells growth factors because they became more sensitive to IL2; but they were also stimulated by a 50-kilodalton (KD) B cell growth factor (BCGF) factor devoid of any T cell proliferation activity. Together these results gave a better characterization of azurophilic granules containing T chronic lymphocytic leukemia, and enabled the documentation of the differentiation of LGL with NK activity.

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A T chronic lymphocytic leukemia with large granular lymphocytes phenotype and functions of leukemic cells under in vitro treatment by differentiation inducers

Merle‐Béral¹,

Boucheix²,

Karray³

et al. 1987

Cancer

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show abstract

Effect of sera from patients with Hodgkin's disease on normal donor lymphocytes containing parallel tubular structures

Smit

Blom

Luyn

et al. 1984

Blut

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Normal donor peripheral blood mononuclear cells incubated for 24 h with sera from patients with Hodgkin's disease were investigated by electron microscopy for the presence of parallel tubular structures (PTS) and/or amorphous electrondense granules (large granular lymphocytes = LGL). In comparison with normal human serum, 14 out of 29 sera of the patients induced a marked increase in the percentage of LGL. From a limited number of experiments it was likely that this increase is paralleled by an increase in Fcgamma receptor-bearing cells after the incubation. This serum effect did not show a correlation with the number of Fcgamma receptor-positive lymphocytes in the peripheral blood of the patients. A difference in the induction effect could be demonstrated between the sera from patients with a favourable and those with an unfavourable clinical course, but this distinction was not absolute. The presence or absence of splenic involvement by Hodgkin's disease does not apparently influence the effect of the sera. From experiments using sera positive for immune complexes or anti-Epstein-Barr virus antibodies, it seems unlikely that these factors are responsible for the observed increase in LGL.

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Lymphocytes with parallel tubular structures: Morphologically a distinctive subpopulation

Smit

Blom

Luyn

et al. 1983

Blut

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Peripheral blood lymphocytes were fractionated in T, B and Null cell enriched subsets by means of sheep red blood cell rosette (ESRBC) sedimentation and nylon wool adherence. The ultrastructural features of these subpopulation were investigated. The T cell fraction in which the sheep erythrocytes were removed from the ESRBC rosette-forming cells (ESRBC-RFC) by lysis with ammonium chloride, consisted mainly of two morphologically distinctive subsets. The majority of the cells (80%) displayed a smooth surface membrane and had a high nuclear to cytoplasmic ratio with few cytoplasmic organelles. The other cell type (18%) had a relatively rough surface membrane, a low nuclear to cytoplasmic ratio, often an indented nucleus and numerous cytoplasmic organelles such as characteristic amorphous granules and sometimes parallel tubular structures (p.t.s.). If the T cells were obtained after mechanical vibration of the ESRBC-RFC, the majority of these cells appeared morphologically identical to this latter cell type. Cells with p.t.s. and amorphous granules were also demonstrated within the Null and B cell enriched fractions (50% and 25% respectively), though in the B cell enriched fraction this cell type is probably due to a contamination of Null cells. Previous observations had already demonstrated that these cells in the three fractions represent the Fc gamma receptor-bearing lymphocytes. The similarities suggest that the Fc gamma receptor-bearing and p.t.s. containing lymphocytes form a morphologically distinct subpopulation.

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Parallel tubular arrays in severe combined immunodeficiency disease: an ultrastructural study of peripheral blood lymphocytes

Cited by 18 publications

References 0 publications

A T chronic lymphocytic leukemia with large granular lymphocytes phenotype and functions of leukemic cells under in vitro treatment by differentiation inducers

A T chronic lymphocytic leukemia with large granular lymphocytes phenotype and functions of leukemic cells under in vitro treatment by differentiation inducers

Effect of sera from patients with Hodgkin's disease on normal donor lymphocytes containing parallel tubular structures

Lymphocytes with parallel tubular structures: Morphologically a distinctive subpopulation

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