Occurrence of circulating immune complexes in beta-thalassaemia major.

Casali, Paolo; Borzini, P; Vergani, Diego; Mieli‐Vergani, G.; Masera, Giuseppe; Zanussi, C

doi:10.1136/adc.53.2.141

Cited by 8 publications

(4 citation statements)

References 5 publications

(6 reference statements)

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“…Serum concentrations of C3 and C4 were found to be normal [2,36,37] or slightly decreased [33,36]. In some patients, a deficiency in the hemolytic activity of the classical [37] or the alternative complement pathway [2] was detected; in contrast, our patients had normal C3 and C4 levels as well as an intact comple ment activity.…”

Section: Discussionmentioning

confidence: 78%

Immunologic Evaluation of Children with Homozygous Beta-Thalassemia Treated with Desferrioxamine

Speer

Gahr²,

Schuff‐Werner³

et al. 1990

Acta Haematol

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In 15 children with thalassemia major (age 4–17 years) a detailed analysis of different immune functions was performed: phagocyte function, specific cellular immunity, humoral defense system. All patients had been subjected to a desferrioxamine therapy and a high transfusion regimen. Examination of neutrophil function included adherence, random migration, chemotaxis, killing of Escherichi coli and production of superoxide radical; these neutrophil functions were shown to be normal. In addition, lymphocyte proliferation in response to different lectins (phytohemagglutinin, concanavalin A, pokeweed mitogen) was identical in patients and controls. However, the number of circulating T-lymphocytes, helper T-cells and B-lymphocytes was increased in some patients. This phenomenon probably reflects an unspecific stimulation of the antibody-producing cells by repeated blood transfusions.

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Section: Discussionmentioning

confidence: 78%

Immunologic Evaluation of Children with Homozygous Beta-Thalassemia Treated with Desferrioxamine

Speer

Gahr²,

Schuff‐Werner³

et al. 1990

Acta Haematol

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“…Such a depletion of suppressor T-cells may contribute to the increased production of immunoglobulin observed in hypertransfused patients (a normal percentage of B-circulating lymphocytes was noted). Casali et al (1978) observed an increase in circulating soluble immunocomplexes in thalassaemics treated with multiple transfusions, related to the age of the patient; complement levels were also decreased. This increase of immunocomplexes seems to be a consequence of the development of chronic hepatitis due to HB virus or to iron overload, or both (Masera and Jean, 1973;Masera et al, 1976).…”

Section: Discussionmentioning

confidence: 87%

Lymphocyte changes in beta-thalassaemia major.

Musumeci¹,

Schilirò²,

Romeo³

et al. 1979

Archives of Disease in Childhood

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SUMMARY Lymphocyte subpopulations were studied in 20 hypertransfused patients with ,B-thalassaemia major, some of whom had been splenectomised. B-lymphocytes were normal but T-lymphocytes were decreased in all patients. The T-cell count was lower in the splenectomised patients than in the nonsplenectomised ones. In the former, the active rosette-forming lymphocytes were also diminished, but the difference was not significant. In all patients the percentage of null cells was greater and the activity of K-cells increased compared with controls.

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“…The longer persistence of antigens in the body increases the anti body response. As the disease evolves, RES iron overload further impairs its function; this favors the formation of circulating immunocomplexes [13], which, in turn, aggra vate the engulfment of RES.…”

Section: Discussionmentioning

confidence: 99%

Serum Immunoglobulins in Homozygous β-Thalassemia

et al. 1981

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IgG, IgA and IgM levels were studied in 187 homozygous β-thalassemic patients and compared with age-matched normal control subjects. The not yet transfused and the polytransfused nonsplenectomized patients showed a significant increase of all Ig classes. The polytransfused splenectomized patients showed a significant increase only of IgG and IgA. The splenectomized patients, when compared to the nonsplenectomized ones, showed a significant increase of IgG, of IgA in the elder ones and a significant reduction of IgM. High ASLO and ASFLO titers were found especially in the splenectomized patients. The high Ig levels in younger not yet transfused patients, with little iron storage and normal hepatic enzyme values, demonstrate that transfusion therapy and liver damage do not play a main role in hypergammaglobulinemia. The absence of antimitochondrial, anticonnective tissue and homogeneous antinuclear antibodies seems to exclude a deficient suppressor mechanism. The hemocatheteric ‘overworking’ of RES may reduce the antigen clearance with a consequent increase of antibody response. Splenectomy, by removing a consistent part of RES, enhances the phenomenon.

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Occurrence of circulating immune complexes in beta-thalassaemia major.

Cited by 8 publications

References 5 publications

Immunologic Evaluation of Children with Homozygous Beta-Thalassemia Treated with Desferrioxamine

Immunologic Evaluation of Children with Homozygous Beta-Thalassemia Treated with Desferrioxamine

Lymphocyte changes in beta-thalassaemia major.

Serum Immunoglobulins in Homozygous β-Thalassemia

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