Beta- poor compliance with the therapy in

Beta-thalassemia major is considered to be one of the most common inheritedhemolytic anemia in Egypt.

Our study showed that the patients were underweightas their BMI was significantly lower compared to that of controls, thisunderweight is in accordance with previous reports and can be explained bydisorder in the endocrine system as a result of iron overload and prolonged useof iron chelating therapy (23, 24). The present significant reduction in Hb,and red blood indices as HCT, MCV, and MCH in our patients compared to controlsmay be attributed to defect in the erythropoiesis associated with beta-thalassemia, similar data was also reported by Filiz Simsek and his colleagues(25). The higher the serum level of iron and ferritin, and the lower serumlevel of TIBC that are found here and in other studies (25, 26) areconsequences of the absence of beta globin chains and accumulation of unpairedalpha globin that causes iron overload and hence cellular oxidative damage.(26)For traceelements, although some studies showed the increased serum Cu levels inpatients with beta-thalassemia major (27-29).

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Others found, reverse results(30-32). We and those who found hypercupraemia in patients withbeta-thalassemia major related that finding to hemochromatosis associate withbeta-thalassemia major.Regarding serumzinc, their levels were found to be significantly lower than controls, that maybe either due to an excessive release from hemolyzed red cells, desferrioxaminetherapy or as of a result of undernutrition in those patients (33, 34).Although several studies revealed lower serum zinc in thalassemia patients (33,34), others revealed higher serum zinc in the thalassemia patients with norelation to ferritin level (35). Our patients also showed a significantly lowerserum Ca and significantly higher phosphorous levels that are in agreement withprevious studies  (8,9,10),  we think that the reason for this might bedelay in starting iron chelating therapy and poor compliance with the therapyin some of our patients and this further explains the underweight in thosepatients. However other reports reported no changes in mean calcium andphosphorous levels (36, 37). Furthermore, serum Mg levels were significantlyreduced in beta-thalassemia patients than in controls this finding was inagreement with Md Fazlul Karim, et al. who explained the presence of hypomagnesemia as a result of lowerthyroid hormones resulted from iron overload (38).

On the other hand, oppositeresult described in Al-Samarrai AH et al study, (28)  Herein, the majority of the patients had a high ferritin levels (mean 1087 ± 519.6ug/l) with more than 50% of patients more than 1000 ug/l, as a result ofrepeated blood transfusion, that is the main feature of beta-thalassemiapatients (39-42). So, we tried to correlate our findings in patients afterdividing them into 2 groups depending on the clinical cut of (1000 ug/l), No,the results didn’t differ significantly with ferritin level ?1000 ug/l. Thisnew finding may be either due to the cut of (1000 ug/l) is very high so thepathology is present in both groups equally.The present workfound T cells but not B cells or NK cells are raised in beta-thalassemiapatients, this indicate activation of the cell-mediated immune cells in thosepatients, furthermore, this increase in the total T lymphocyte pool is mainlyrelated to increase in the cytotoxic T lymphocytes and not in T helperlymphocytes, that in fact were reduced in our patients.

This is in accordancewith the concept described by many other researchers (43- 45). Whether thisspecific increase in CD8 T cells and the decrease CD4 is due to continuousalloantigenic stimulation of the immune system with autoimmune hemolysis as aresult of iron overload remain to be elusive. The different finding by otherswho found either increase in the proportion of both CD8 positive cells and CD4positive cell or no change in T lymphocyte population (12, 46).  The discrepancy between our results andtheirs may be related to the changes in the immune system is time-related anddepending on the disease duration and iron overload amount. The absence ofchanges in the other immune arms (humoral and innate) reported here came inagreement with Ahmadiafshar’ s finding who found no abnormalities in cellularand humeral system (47) and unlike those who found alteration ofimmunoglobulins (48). Further analysis of the effect of ferritin on immunemarkers depending on the cut off 1000 ?g/L could reveal  significant difference between patients withferritin level ?1000 and patients with ferritin <1000 when compared with thecontrols, as the level of T cells and CD8 positive cells is higher in patientswith ferritin level ?1000 but not in patients with ferritin <1000, inagreement with this a recent work of Hagag and others (44,45) found significanta negative correlation between serum ferritin and CD3 and CD4, and positivecorrelation with CD8.

Our results were in contrast with. Noulsri et al (49) whoreported insignificant differences in T-cell subsets CD3, CD4 and CD8 betweenpatients and controls and they concluded that high iron levels inbeta-thalassemic patients have a more significant effect on the function and activityof T cells rather than on their number. (46,49). Variation among results can becontributed to several factors as, the clinical heterogeneity among beta-thalassemia patients, frequency of blood transfusion, spelenctomy, serum ironstatus, iron chelation therapy  Inconclusion, although the alteration in the level of trace elements and cellmediated immunity did not significantly differ among beta- thalassemia patientswith  high ferritin level (?1000) andthose with low ferritin level (<1000), the impaired levels of thesemolecules and cells in the whole patients indicates oxidative stress anddamage  even in patients with ferritinlevel (<1000), initiating chelation therapy regardless of the level offerritin can be a more realistic approach in beta-thalassemia major,  in addition, further large scale studies are recommended.


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