Natural Killer Cell Activity and T Subpopulations in Thalassemia major

Neri, Anthony; Brugiatelli, Maura; Iacopino, Pasquale; Callea, Vincenzo; Ronco, Francesca

doi:10.1159/000206598

Cited by 16 publications

(8 citation statements)

References 18 publications

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“…Lastly, and interest ingly, the present study supports those investigations that indicate an immunodepressive effect of blood transfu sion. A depressed function of NK cells [12,13], T lym phocyte subsets abnormalities [13,[15][16][17], and decreased in vitro response of T cells to mitogens [14,17] have been reported in the literature and the two former abnormali ties were confirmed by the present study. While some of the above in vitro abnormalities can be induced by con comitant factors, blood transfusion shows immunode pressive effects also at the clinical level.…”

Section: Resultssupporting

confidence: 86%

The Clinical Importance of Leukocyte Depletion in Regular Erythrocyte Transfusions

Sirchia¹,

Rebulla²,

Mascaretti³

et al. 1986

Vox Sanguinis

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Antilymphocyte, antigranulocyte and antiplatelet alloantibodies, T lymphocyte subsets, expression of HLA-DR antigens on T lymphocytes and NK cell function were determined in 11 homozygous β-thalassemic children multitransfused ab initio with Erypur-filtered leukocyte-free red cell units (group A) and in 13 similar children multitransfused with standard packed red cell units (group B). No antibodies were found in group A patients, whereas 69% of group B patients were immunized. The two groups did not differ significantly with regard to the other test results. Considered together, thalassemia patients showed a percentage of T4+ cells and a NK cell function that were significantly lower than those found in a reference group of 16 healthy male blood donors. Thalassemics moreover showed a higher than normal percentage of T3+, T4+ and T8+ cells expressing HLA-DR antigens. The results indicate that leukocyte-free red cells should be the treatment of choice for prospective recipients of multiple transfusions, since they are capable of preventing (or delaying) the production of alloantibodies against leukocytes and platelets. From the data of the present study, it does not seem that the transfusion of leukocyte-free red cells is capable of preventing the abnormalities of some immunological tests that occur in some multitransfused patients. Further investigations, however, are needed to draw conclusions on this problem.

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Section: Resultssupporting

confidence: 86%

The Clinical Importance of Leukocyte Depletion in Regular Erythrocyte Transfusions

Sirchia¹,

Rebulla²,

Mascaretti³

et al. 1986

Vox Sanguinis

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“…They include: impaired activity of monocytes and neutrophils [4, 5], defective activity of the complement alternative pathway [6], increased polyclonal serum immunoglobulin levels [1, 2, 3], numerical or functional alterations of different peripheral blood lymphocyte subpopulations [1, 2, 3, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18]and anomalies of serum levels of cytokines and soluble antigens [3]. The pathogenic mechanism of these abnormalities is not clarified.…”

Section: Introductionmentioning

confidence: 99%

Immunological Evaluation of Patients with Beta-Thalassemia major

Consolini

Calleri²,

Legitimo³

et al. 2001

Acta Haematol

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Abnormalities in the immune system and zinc homeostasis in patients with β-thalassemia major (TM) have been reported. Since zinc ion is essential for the efficiency of the immune system and is required to induce biological activity to thymulin (Zn-FTS), a biochemically defined thymic hormone, we investigated the plasma levels of zinc and both active thymulin (Zn-FTS) and total zinc saturable thymulin (Zn-FTS+FTS) in 18 patients with TM aged between 2 and 31 years and 22 normal controls of the same age. Inhibitory molecules anti-thymulin and the distribution of lymphocyte subsets were also analyzed. Patients with TM presented significantly lowered plasma zinc and thymulin levels when compared to normal subjects. The significant enhancement of the active form of the hormone after zinc addition in vitro suggests that low thymulin values found in TM are due not to a thymic failure in synthesizing and secreting the thymic hormone, but a defect in zinc saturation of the hormone. An impairment of cell subset distribution was also demonstrated. This study shows that zinc and thymulin deficiency contribute to the complex mechanisms underlying immune dysfunction in TM.

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“…Transfusions lead to iron overload and also to immune derangements, both of which exert a negative effect on the functional integrity of the immune system in multitransfused patients with thalassaemia. There are already a large number of reports describing immune abnormalities in thalassaemia, namely defective function of polymorphonuclear neutrophils and monocytes [2,3], decrease of CD4 + cells and increase of CD8 + cells [4][5][6], diminished mitogen responses [5] and low natural killer (NK) cell activity [7,8]. These and probably other undetected abnormalities may explain the tendency for severe or unusual infections.…”

Section: Introductionmentioning

confidence: 99%

A distinct pattern of cytokine production from blood mononuclear cells in multitransfused patients with β-thalassaemia

Salsaa

Zoumbos

1997

Clinical and Experimental Immunology

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SUMMARYThe unstimulated and induced production of granulocyte-macrophage colony-stimulating factor (GM-CSF), granulocyte colony-stimulating factor (G-CSF), IL-3, IL-6, stem cell factor (SCF), IL-1b, tumour necrosis factor-alpha (TNF-a), TNF-b, interferon-gamma (IFN-g) and transforming growth factor-beta (TGF-b) was determined after culture of blood mononuclear cells from 22 patients with severe b-thalassaemia in a regular transfusion programme, five non-regularly transfused patients with b-thalassaemia intermedia and nine normal persons. A distinct pattern of cytokine production in thalassaemic patients was detected, namely a low unstimulated production of all cytokines and a significant increase in the stimulated production of IFN-g, TNF-a and IL-1b; these abnormalities were more pronounced in the more heavily transfused older patients. The increased production of the above cytokines, which usually characterize the acute response to infectious agents and have a negative effect on erythropoiesis, may explain the deterioration of anaemia found in thalassaemic patients during acute infections.

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Natural Killer Cell Activity and T Subpopulations in Thalassemia major

Cited by 16 publications

References 18 publications

The Clinical Importance of Leukocyte Depletion in Regular Erythrocyte Transfusions

The Clinical Importance of Leukocyte Depletion in Regular Erythrocyte Transfusions

Immunological Evaluation of Patients with Beta-Thalassemia major

A distinct pattern of cytokine production from blood mononuclear cells in multitransfused patients with β-thalassaemia

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