G. Lombardi et al., SERUM LEVELS OF CYTOKINES AND SOLUBLE-ANTIGENS IN POLYTRANSFUSED PATIENTS WITH BETA-THALASSEMIA MAJOR - RELATIONSHIP TO IMMUNE STATUS, Haematologica, 79(5), 1994, pp. 406-412
Background. A series of immunological abnormalities has been described
in patients with beta-thalassemia The aim of this study was to invest
igate whether the measurement of serum levels of selected cytokines an
d soluble molecules (deriving from cell membrane antigens) involved in
the immune response could be useful for a better definition of such a
lterations. Patients and Methods. Serum levels of interleukin-2 (IL-2)
, IL-6, tumor necrosis factor (TNF), soluble (s) CD4, sCD8, sCD23 and
sCD25 were measured using immunoenzymatic assays in 45 transfusion-dep
endent patients affected by beta-thalassemia major and correlated to c
onventional immunological indexes, such as peripheral lymphocyte subpo
pulations and circulating immunoglobulins. Results. Patients with beta
-thalassemia major showed increased TNF, sCD8, sCD23 and sCD25 and low
er sCD4 values compared to normal controls. IL-2 and IL-6 were found t
o be undetectable or within the normal range in all patients. Splenect
omized patients presented lower levels of sCD8 and sCD23 than those ob
served in unsplenectomized ones. A series of correlations involving TN
F, sCD8, sCD23, sCD25, serum immunoglobulins and some lymphocyte subpo
pulations was observed. In addition, serum markers of immune activatio
n (TNF, sCD23, sCD25) correlated directly with the annual blood transf
usion requirement. Despite this series of immunological anomalies, no
patient had a history of repeated infectious episodes. Conclusions. Po
lytransfused beta-thalassemic patients are characterized by a partial
functional immunodeficiency determined by increased activity of CD8+ s
uppressor/cytotoxic lymphocytes and possibly reduced activity of the C
D4+ helper/inducer subset. B-lymphocytes also appear highly activated.
The allo-antigenic stimulation of transfusions seems to play a major
role in the determination of these defects; however, this functional i
mmunological imbalance does not seem to have any clinical relevance.