Influencing factors of iron metabolism assessment in patients with myelodysplastic syndrome: A retrospective study / 中华血液学杂志

Objective: To analyze the influencing factors of iron metabolism assessment in patients with myelodysplastic syndrome. Methods: MRI and/or DECT were used to detect liver and cardiac iron content in 181 patients with MDS, among whom, 41 received regular iron chelation therapy during two examinations. The adjusted ferritin (ASF) , erythropoietin (EPO) , cardiac function, liver transaminase, hepatitis antibody, and peripheral blood T cell polarization were detected and the results of myelofibrosis, splenomegaly, and cyclosporine were collected and comparative analyzed in patients. Results: We observed a positive correlation between liver iron concentration and ASF both in the MRI group and DECT groups (r=0.512 and 0.606, respectively, P<0.001) , only a weak correlation between the heart iron concentration and ASF in the MRI group (r=0.303, P<0.001) , and no significant correlation between cardiac iron concentration and ASF in the DECT group (r=0.231, P=0.053) . Moreover, transfusion dependence in liver and cardiac [MRI group was significantly associated with the concentration of iron in: LIC: (28.370±10.706) mg/g vs (7.593±3.508) mg/g, t=24.30, P<0.001; MIC: 1.81 vs 0.95, z=2.625, P<0.05; DECT group: liver VIC: (4.269±1.258) g/L vs (1.078±0.383) g/L, t=23.14, P<0.001: cardiac VIC: 1.69 vs 0.68, z=3.142, P<0.05]. The concentration of EPO in the severe iron overload group was significantly higher than that in the mild to moderate iron overload group and normal group (P<0.001) . Compared to the low-risk MDS group, the liver iron concentration in patients with MDS with cyclic sideroblasts (MDS-RS) was significantly elevated [DECT group: 3.80 (1.97, 5.51) g/L vs 1.66 (0.67, 2.94) g/L, P=0.004; MRI group: 13.7 (8.1,29.1) mg/g vs 11.6 (7.1,21.1) mg/g, P=0.032]. Factors including age, bone marrow fibrosis, splenomegaly, T cell polarization, use of cyclosporine A, liver aminotransferase, and hepatitis antibody positive had no obvious effect on iron metabolism. Conclusion: There was a positive correlation between liver iron concentration and ASF in patients with MDS, whereas there was no significant correlation between cardiac iron concentration and ASF. Iron metabolism was affected by transfusion dependence, EPO concentration, and RS.

Naringin is a promising natural compound for therapy of iron-overload disorders

Abstract Naringin has been shown to exhibit satisfying iron chelation capacity. Considering the side effects of routinely-used iron chelator (desferrioxamine, DFO), we decided to evaluate the iron chelation potency of naringin to discover whether or not it can be a promising natural substitute for treatment of excessive iron-related diseases. 35 mice were classified into five groups of 7 and subjected to iron dextran administration to induce the iron-overload condition. Iron-overloaded mice were then treated with normal saline (as control), naringin or DFO Morphology changes, and iron deposition in liver tissues were studied using H&E and Perl's staining. The results revealed that naringin is more potent than DFO in removing excessive iron ions deposited in liver tissues, indicating that naringin is a promising natural compound for therapy of iron overload disorders

Beta Thalassemia Major with Diabetes Mellitus: A Case Report

Thalassemia occurs due to defects in normal hemoglobin production. Globally it is most common inherited anaemia. Diabetes is a complication of b-thalassemia major. We report a case of Diabetes mellitus in a known case of beta thalassemia major. Patient had undergone splenectomy 1 year back. Patient is taking chelating agent Defasirox 1000mg orally once a day in the morning. Family history reveals, born through third degree consanguineous marriage. The patient was then subjected for laboratory examination reveals BSLwas 490, urine ketone 2+, urine sugar 3+, ABG was normal, HbA1c was 13 & 3 month old report of serum ferritin 1200 ng/dl. TFTand GH studies normal. Multidisciplinary management was instituted. Blood sugar level got controlled over subcutaneous insulin. Patient may have landed in Diabetic ketoacidosis but was promptly diagnosed & treated. This case is presented for its rarity. Due to increase in life expectancy of patient with thalassemia major, patient will expose more years of hyperglycemia and diabetes. Sustaining metabolic control and controlling cardiovascular risk factors helps to prevent future complications.

Neuroprotective effects of kukoamine A on 6-OHDA-induced Parkinson's model through apoptosis and iron accumulation inhibition / 中草药·英文版

Objective: Parkinson's disease (PD) is characterized by the loss of dopaminergic neurons in substantia nigra (SN). Our previous study demonstrated kukoamine A (KuA) to exhibit strong neuroprotective effects through antioxidative stress, and autophagy in MPTP/MPP

Hearing Loss in Thalassemic Children on Chelation Therapy

Background: Thalassemia is a common genetic hematological disorder worldwide. It is also common in North India includingJammu region. These patients need lifelong repeated blood transfusions and iron chelation therapy for their survival. Chelationtherapy is known to be associated with various complications including sensorineural hearing loss (SNHL). Till now, no dataare available regarding SNHL in pediatric thalassemia major patients in Jammu region. Hence, we planned a study to assessthe prevalence of hearing loss in children with thalassemia major in the age group of 10–20 years.Methods: All the children with beta-thalassemia major in the age group of 10–20 years registered with Thalassemia Day CareCenter, Department of Pediatrics, SMGS Hospital, Government Medical College, Jammu, were enrolled in this cross-sectionalstudy. Hearing was assessed by pure tone audiometry. Clinical and demographic data of these patients were recorded onpretested pro forma and analyzed.Results: A total number of 34 children with thalassemia in the age group of 10–20 years were enrolled in this study whichcomprised 18 males and 16 females. Out of these 34 patients, 5 (14.7%) were found to have SNHL and 1 (2.9%) had conductivehearing loss. Four of the five patients in SNHL group had low- as well as high-frequency mild hearing loss (25–40 db) whileone patient had high-frequency mild hearing loss at 4000 HZ. Four out of these five patients had unilateral hearing loss on theleft side while one had bilateral SNHL. Two out of five patients in the SNHL group were taking chelation therapy in the form ofcombination of deferiprone and deferasirox at the dose of 75–100 mg/kg/day and 30–40 mg/kg/day, respectively, for more than5 years. The other three patients were taking only deferasirox at the dose of 30–40 mg/kg/day for more than 5 years.Conclusions: Regular blood transfusions and chelation therapy are essential for long-term survival of thalassemia majorpatients but are also associated with complications like SNHL.

Knowledge, attitude and practice in parents of chronically transfused thalassemic patients regarding thalassemia in thalassemia day care unit in government medical college, Amritsar, Punjab, India

Background: Thalassemia is a chronic debilitating disease that affects nearly 200 million people worldwide. A caregiver who has good knowledge regarding the disease can not only provide a better quality of care to his/her ward but also may spread knowledge in the society in which he/she lives which helps immensely in raising community awareness related to the disease.Methods: 50 caregivers (either mother or father) of chronically transfused thalassemic children were questioned regarding their knowledge, attitude and practice towards this disease, using a self-constructed questionnaire.Results: It was found that despite adequate knowledge regarding every aspect of this disease, there is a lack of attitude of practice towards prevention of this disease in their subsequent child or in their near and dear ones.Conclusions: There is a need to promote an attitude of practice in prevention of the birth of thalassemic children as mere knowledge regarding the disease is not enough in the present scenario where the disease burden is increasing as is the financial and emotional burden on the families. Role of Government sponsorship, a social worker and a child psychologist in the Thalassemia day care unit cannot be minimized.

Impacts of transfusion in β-thalassemia major patients receiving multiple blood transfusions

Background: Beta-thalassemia major is one of major public health problems in India. Thalassemia major is a transfusion-dependent severe anemia and these children experience various problems if the transfusion is inadequate but at the same time repeated blood transfusions are associated with hazards like iron overload.Methods: This study was conducted at thalassemia ward of S.M.S Hospital and J.K.LON paediatrics Hospital, Jaipur from April 2012 to Nov. 2013. 145 thalassemia patients on transfusion therapy attending outdoor or being admitted were assessed after obtaining due permission from the authorities and consent from the guardian or parents of patients.Results: Present study comprised 145 beta-thalassemia major patients with 51 (35%) females and 94 (65%) males in which youngest patient is 3 years old and the oldest 33 years. 104 (72%) were Hindus and 41 (28%) were Muslims. Consanguinity was found in a significant proportion 16% (23) of the parents of the patients. 137 (94%) patients had serum ferritin ³1000ng/ml. Out 145 thalassemic patients, 108 (74.5%) have total serum bilirubin >1(mg/dl), 103 (71%) have SGPT level >35IU, 103 (71%) have SGOT level >40IU), 38 (26.2%) have serum alkaline phosphatase level >390 IU, 35 (24.1%) have serum creatinine level >1.6mg/dl, 42 (29%) have serum urea level >45mg/dl, 28 (19.3%) have serum uric acid level >6mg/dl.Conclusions: It is suggested to revise and devise suitable transfusion regime so that a balance between adequate transfusion and minimum side effects of multiple transfusions is maintained. Systemic effects of multiple transfusions should be rigorously and meticulously studied.

Evaluation Of Free Radical Scavenging Ability And Antiradical Activities Of Ximenia Caffra Fruit Extracts At Different Ripening Stages

Objective: To evaluate the free radical scavenging ability and antiradical activities of Ximenia caffra fruit extracts in their different ripening stages. Methods: Using standard procedures, Ximenia caffra fruit extracts were determined for ferrous ions chelating ability, nitric oxide and hydroxyl radical scavenging ability. Antiradical activities were assessed using 1,1-diphenyl-2-picrylhydrazyl (DPPH) in which the fruit extracts were evaluated for the number of antiradical units (AU515), the number of antiradical units per 1 mg of extracts (EAU515) and the total number of antiradical units per 1g of raw fruits (TAU515). Results: The fruit extracts exhibited significant higher ferrous ion chelating and free radical scavenging capacity compared to synthetic antioxidants (standard). Fruit extracts in early ripening stage (ERS) exhibited stronger ion chelation, nitric oxide and hydroxyl radical scavenging ability with low effective fruit extract concentration required to reduce free radicals by 50% (EC50) that were 14, 25 and 30µg/ml respectively than the late ripening stage (LRS) extracts. The fruit extracts also showed high AU515, EAU515 and TAU515. The values of AU515 ranged from 0.93 to 0.95, while EAU515 from 78.30 to 79.34 and TAU515 ranged from 19762.46 to 23821.23 of the extracts in LRS and ERS respectively. Conclusion: Based on these observations Ximenia caffra is potentially beneficial to human health due to its strong ability to scavenge free radicals. Its utilization can potentially reduce the risk of degenerative diseases to human beings.

Study of iron overload assessment by T2* magnetic resonance imaging in patients with myelodysplastic syndromes / 中华血液学杂志

Objectives@#To analyze the cardiac T2* value, liver iron concentration (LIC) , and related laboratory parameters in myelodysplastic syndrome (MDS) with iron overload and evaluate the changes of organ functions after iron chelation therapy. To explore the value of magnetic resonance imaging (MRI) T2* in making early diagnosis and assessing organs iron overload.@*Methods@#Retrospective investigation was used to observe the cardiac T2* value, LIC, iron metabolism parameters and related laboratory parameters of 85 MDS patients from Nov 2014 to Jan 2018. Among them, 7 MDS patients with Low/Int-1 have received iron chelation therapy for 6 months during two MRI examinations. The above parameters were collected before and after iron chelation therapy for comparison.@*Results@#Correlations were found between heart T2* value and age (rs=-0.290, P=0.007) and left ventricular ejection fraction (LVEF) (rs=0.265, P=0.009) . There was a significant negative correlation between heart T2* value and blood transfusion units (rs=-0.701, P<0.001) . There was a significant positive correlation between LIC and serum ferritin (SF) (rs=0.577, P<0.001) . There was also a correlation between LIC and ALT (rs=0.268, P=0.014) and blood transfusion units (rs=0.244, P=0.034) . There was no correlation between heart T2* and pro-BNP, SF (all P>0.05) , and no correlation between LIC and age (P>0.05) . The increase of heart T2* between the normal and abnormal groups was statistically significant (P=0.005) , but the iron overload ratio of the heart T2*<20 ms was not significant between the two groups. There was statistical significance in the proportion of severe liver iron overload (LIC>15 mg/g DW) (P=0.045) . After iron chelation therapy, the values of SF, transferrin saturation, ALT, AST, pro-BNP and LIC of 7 patients were decreased compared with values before iron chelation therapy, and the peripheral blood cell level was increased. However, the changes of LVEF and T2* values after iron chelation were not obvious.@*Conclusion@#MRI T2* may be a predictor of iron overload in patients with MDS in early stage, and may be more valuable compare with LVEF, SF and other laboratory indicators. The safety and repeatability of MRI cardiac T2* examination are recognized, and it can be used as an ideal detection for patients with iron overload.

Study of iron overload assessment by T2* magnetic resonance imaging in patients with myelodysplastic syndromes / 中华血液学杂志

Objectives: To analyze the cardiac T2* value, liver iron concentration (LIC) , and related laboratory parameters in myelodysplastic syndrome (MDS) with iron overload and evaluate the changes of organ functions after iron chelation therapy. To explore the value of magnetic resonance imaging (MRI) T2* in making early diagnosis and assessing organs iron overload. Methods: Retrospective investigation was used to observe the cardiac T2* value, LIC, iron metabolism parameters and related laboratory parameters of 85 MDS patients from Nov 2014 to Jan 2018. Among them, 7 MDS patients with Low/Int-1 have received iron chelation therapy for 6 months during two MRI examinations. The above parameters were collected before and after iron chelation therapy for comparison. Results: Correlations were found between heart T2* value and age (rs=-0.290, P=0.007) and left ventricular ejection fraction (LVEF) (rs=0.265, P=0.009) . There was a significant negative correlation between heart T2* value and blood transfusion units (rs=-0.701, P<0.001) . There was a significant positive correlation between LIC and serum ferritin (SF) (rs=0.577, P<0.001) . There was also a correlation between LIC and ALT (rs=0.268, P=0.014) and blood transfusion units (rs=0.244, P=0.034) . There was no correlation between heart T2* and pro-BNP, SF (all P>0.05) , and no correlation between LIC and age (P>0.05) . The increase of heart T2* between the normal and abnormal groups was statistically significant (P=0.005) , but the iron overload ratio of the heart T2*<20 ms was not significant between the two groups. There was statistical significance in the proportion of severe liver iron overload (LIC>15 mg/g DW) (P=0.045) . After iron chelation therapy, the values of SF, transferrin saturation, ALT, AST, pro-BNP and LIC of 7 patients were decreased compared with values before iron chelation therapy, and the peripheral blood cell level was increased. However, the changes of LVEF and T2* values after iron chelation were not obvious. Conclusion: MRI T2* may be a predictor of iron overload in patients with MDS in early stage, and may be more valuable compare with LVEF, SF and other laboratory indicators. The safety and repeatability of MRI cardiac T2* examination are recognized, and it can be used as an ideal detection for patients with iron overload.

Comparative Efficacy and Safety of Oral Iron Chelators and their Novel Combination in Children with Thalassemia.

Objective: To compare the efficacy and safety of oral iron chelators (Deferiprone and Deferasirox) when used singly and in combination in multi-transfused children with thalassemia. Design: Prospective comparative study. Setting: Thalassemia Center of a medical college affiliated hospital Participants and Intervention: 49 multi-transfused children with thalassemia with a mean (SD) age 11.6 (6.21) y received daily chelation therapy with either deferiprone alone (75 mg/kg/day in 3 divided doses), deferasirox alone (30 mg/kg/day single dose) or their daily combination (same dose as monotherapy) for 12 months. Outcome measures: Serum ferritin levels at the start of study, after 6 months and after 12 months. MRI T2* of liver and heart initially and after 6 months of follow up. 24-hour urinary iron excretion values at the outset and after 12 months of chelation therapy. At every visit for blood transfusion, all patients were clinically assessed for any adverse effects; liver and renal functions were monitored 6-monthly. Results: After 12 months of respective chelation therapy, serum ferritin values decreased from a mean of 3140.5 ng/mL to 2910.0 ng/mL in deferiprone alone group, 3859.2 ng/mL to 3417.4 ng/mL in deferasirox alone group and from 3696.5 ng/mL to 2572.1 ng/mL in the combination group. The combination therapy was more efficacious in causing fall in serum ferritin levels compared to deferiprone and deferasirox monotherapy (P=0.035 and 0.040, respectively). Results of MRI T2* were equivocal. Combined drug usage produced maximum negative iron balance in the body by maximally increasing the iron excretion in urine from 61.1 µmol/day to 343.3 µmol/day (P=0.002). No significant adverse reactions were noticed in either the monotherapy or the combination group. Conclusion: Oral combination therapy of deferiprone and deferasirox appears to be an efficacious and safe modality to reduce serum ferritin in multi-transfused children with thalassemia.

Antioxidant potential and oxidative DNA damage preventive activity of unexplored endemic species of Curcuma.

Free radical scavenging activity, ferrous ion chelating capacity, reducing power and genoprotective effect of the aqueous leaf extracts of four unexplored endemic Curcuma spp. (C. vamana, C. neilgherrensis, C. mutabilis, C. haritha) were found to be dose-dependent and were highest in C. vamana. DNA protection property of the extracts was evaluated against H2O2/UV-induced oxidative damage. DNA-methyl green displacement assay showed that these extracts were free of DNA intercalating compounds. Further, hemolysis assay also showed that the extracts were non-toxic to human erythrocytes. The results highlight C. vamana as a promising source for herbal preparations possessing high antioxidant potential and genoprotective activity.

Effect of a novel oral active iron chelator: 1-(N-acetyl-6-aminohexyl)-3-hydroxy-2-methylpyridin-4-one (CM1) in iron-overloaded and non-overloaded mice

Objective: To evaluate efficacy and toxicity of a novel orally active bidentate iron chelator, 1-(N-acetyl-6-aminohexyl)-3-hydroxy-2-methylpyridin-4-one (CM1) in mice under normal and iron overload conditions. Methods: Wild type C57BL/6 mice were fed with normal and 0.2% (w/w) ferrocene-supplemented (Fe) diets, respectively for 240 d and orally given the CM1 (50, 100 and 200 mg/kg) for 180 d. Blood iron profiles, hematological indices, liver enzymes and histopathology were determined. Results: CM1 treatment lowered plasma levels of labile plasma iron and non-transferrin bound iron, but not ferritin in the Fe-fed mice. However, the treatment did not impact blood hemoglobin level, white blood cell and platelet numbers in both normal diet and Fe diet-fed mice. Interestingly, CM1 treatment did not markedly elevate plasma aspartate aminotransferase, alanine aminotransferase and alkaline phosphatase activities in the normal diet-fed mice but it tended to increase the levels of the liver enzymes slightly in the Fe-fed mice. Hematoxylin and eosin staining result showed no abnormal pathological changes in heart, liver and spleen tissues. Conclusions: It is clear that CM1 would not be toxic to bone marrow and liver cells under normal and iron-overload conditions.

Effect of a novel oral active iron chelator: 1-(N-acetyl-6-aminohexyl)-3-hydroxy-2-methylpyridin-4-one (CM1) in iron-overloaded and non-overloaded mice

OBJECTIVE@#To evaluate efficacy and toxicity of a novel orally active bidentate iron chelator, 1-(N-acetyl-6-aminohexyl)-3-hydroxy-2-methylpyridin-4-one (CM1) in mice under normal and iron overload conditions.@*METHODS@#Wild type C57BL/6 mice were fed with normal and 0.2% (w/w) ferrocene-supplemented (Fe) diets, respectively for 240 d and orally given the CM1 (50, 100 and 200 mg/kg) for 180 d. Blood iron profiles, hematological indices, liver enzymes and histopathology were determined.@*RESULTS@#CM1 treatment lowered plasma levels of labile plasma iron and non-transferrin bound iron, but not ferritin in the Fe-fed mice. However, the treatment did not impact blood hemoglobin level, white blood cell and platelet numbers in both normal diet and Fe diet-fed mice. Interestingly, CM1 treatment did not markedly elevate plasma aspartate aminotransferase, alanine aminotransferase and alkaline phosphatase activities in the normal diet-fed mice but it tended to increase the levels of the liver enzymes slightly in the Fe-fed mice. Hematoxylin and eosin staining result showed no abnormal pathological changes in heart, liver and spleen tissues.@*CONCLUSIONS@#It is clear that CM1 would not be toxic to bone marrow and liver cells under normal and iron-overload conditions.

Recent advances in treatment of aplastic anemia

Recent advances in the treatment of aplastic anemia (AA) made most of patients to expect to achieve a long-term survival. Allogeneic stem cell transplantation (SCT) from HLA-matched sibling donor (MSD-SCT) is a preferred first-line treatment option for younger patients with severe or very severe AA, whereas immunosuppressive treatment (IST) is an alternative option for others. Horse anti-thymocyte globuline (ATG) with cyclosporin A (CsA) had been a standard IST regimen with acceptable response rate. Recently, horse ATG had been not available and replaced with rabbit ATG in most countries. Subsequently, recent comparative studies showed that the outcomes of patients who received rabbit ATG/CsA were similar or inferior compared to those who received horse ATG/CsA. Therefore, further studies to improve the outcomes of IST, including additional eltrombopag, are necessary. On the other hand, the upper age limit of patients who are able to receive MSD-SCT as first-line treatment is a current issue because of favorable outcomes of MSD-SCT of older patients using fludarabine-based conditioning. In addition, further studies to improve the outcomes of patients who receive allogeneic SCT from alternative donors are needed. In this review, current issues and the newly emerging trends that may improve their outcomes in near futures will be discussed focusing the management of patients with AA.

Multiple High-affinity Iron-uptake Systems of Vibrio vulnificus

Vibrio vulnificus is a Gram-negative halophilic bacterium that causes necrotizing wound infections and fatal septicemia, which mainly occur in patients with elevated serum or tissue iron levels. Accumulated experimental data clearly show that V. vulnificus is a ferrophilic bacterium that requires more available iron for growth than other pathogenic bacteria, has multiple iron-uptake systems, which play important roles in the pathogenesis of the V. vulnificus infections. This review summarized the composition, regulation and significance of V. vulnificus iron-uptake systems. These iron-uptake systems may be attractive candidates for the development of V. vulnificus vaccine. Iron-chelating therapy can also be a promising modality for the prevention and treatment of V. vulnificus infections.

Carbohydrate content and antioxidative potential of the seed of three edible indica rice (Oryza sativa L.) cultivars.

Rice (Oryza sativa L.) grains or seeds are known to lose much of their nutrient and antioxidant contents, following polishing. The current study was undertaken to evaluate and compare the carbohydrate content and antioxidant parameters in the unpolished and polished seeds of three edible indica rice cultivars, namely Swarna (SW), the most popular indica rice cultivar in India and aromatic or scented cultivars Gobindobhog (GB) and Pusa Basmati (PB). While both the sucrose and starch content was the maximum in PB seeds (both unpolished and polished), the amylose content was the highest in SW polished seeds. SW polished seeds were superior as compared to GB and PB cultivars in terms of total antioxidant capacity, DPPH radical scavenging and Fe(II) chelation potential, as well as the highest lipoxygenase (LOX) inhibition or H2O2 scavenging potential, probably due to the maximum accumulation of total phenolics and flavonoids, the two important antioxidants. The reducing power ability was, however, identical in both SW and GB polished seeds. The PB polished seeds were more potent in superoxide and hydroxyl scavenging, whereas GB in nitric oxide (NO) scavenging. The common observation noted after polishing of seeds was the reduction in the level of carbohydrates and antioxidant potential, though the extent of reduction varied in the three cultivars. The only exception was GB, where there was no alteration in NO scavenging potential even after polishing. Our study showed the better performance of SW polished seeds with respect to higher amylose content and majority of the tested parameters governing antioxidant capacity and radical scavenging potential, thus highlighting the greater dietary significance of SW over the other two cultivars.

In vitro Studies of Iron Chelation Activity of Purified Active Ingredients Extracted from Triticum aestivum Linn. (Wheat Grass).

Objective: Seven to eight days germinated plants of Triticum aestivum (wheat grass) are a rich source of vitamin A, C, calcium, magnesium, phosphorus, potassium, sodium, sulphur, cobalt, zinc and protein. Traditionally the aqueous extract of T. aestivum was reported to be used as a health tonic in folk and ayurvedic medicine. We previously reported that aqueous extract of T. aestivum was found to reduce the blood transfusion requirement in iron overloaded Thalassemia and Myelodysplastic syndrome patients. Our objective was to extract and purify active ingredients from wheat grass and study their mode of action in stabilizing hemoglobin level in those patients. Design and Method: Active ingredients of wheat grass were extracted and purified by cation exchange column chromatography followed by High Performance Liquid Chromatography. In vitro experiments with phenylhydrazine treated red blood cell hemolysate were carried out before and after treatment of purified fraction of T. aestivum to study iron chelating activity. Result: Purified fraction of T. aestivum treated red blood cell showed significant inhibition of free reactive iron production and formation of thio-barbituric acid reactive substances when compared to desferrioxamine treated hemolysate. Conclusion: Iron chelating activity of purified fraction of T. aestivum, an inexpensive, easily available source, is very promising for further clinical trial and development of oral iron chelator drug for Thalassemia, Myelodysplastic syndrome and other iron overloaded diseases.

Iron Overload during Follow-up after Tandem High-Dose Chemotherapy and Autologous Stem Cell Transplantation in Patients with High-Risk Neuroblastoma

Multiple RBC transfusions inevitably lead to a state of iron overload before and after high-dose chemotherapy and autologous stem cell transplantation (HDCT/autoSCT). Nonetheless, iron status during post-SCT follow-up remains unknown. Therefore, we investigated post-SCT ferritin levels, factors contributing to its sustained levels, and organ functions affected by iron overload in 49 children with high-risk neuroblastoma who underwent tandem HDCT/autoSCT. Although serum ferritin levels gradually decreased during post-SCT follow-up, 47.7% of the patients maintained ferritin levels above 1,000 ng/mL at 1 yr after the second HDCT/autoSCT. These patients had higher serum creatinine (0.62 vs 0.47 mg/mL, P = 0.007) than their counterparts (< 1,000 ng/mL). Post-SCT transfusion amount corresponded to increased ferritin levels at 1 yr after the second HDCT/autoSCT (P < 0.001). A lower CD34+ cell count was associated with a greater need of RBC transfusion, which in turn led to a higher serum ferritin level at 1 yr after HDCT/autoSCT. The number of CD34+ cells transplanted was an independent factor for ferritin levels at 1 yr after the second HDCT/autoSCT (P = 0.019). Consequently, CD34+ cells should be transplanted as many as possible to prevent the sustained iron overload after tandem HDCT/autoSCT and consequent adverse effects.

Iron Overload during Follow-up after Tandem High-Dose Chemotherapy and Autologous Stem Cell Transplantation in Patients with High-Risk Neuroblastoma

Multiple RBC transfusions inevitably lead to a state of iron overload before and after high-dose chemotherapy and autologous stem cell transplantation (HDCT/autoSCT). Nonetheless, iron status during post-SCT follow-up remains unknown. Therefore, we investigated post-SCT ferritin levels, factors contributing to its sustained levels, and organ functions affected by iron overload in 49 children with high-risk neuroblastoma who underwent tandem HDCT/autoSCT. Although serum ferritin levels gradually decreased during post-SCT follow-up, 47.7% of the patients maintained ferritin levels above 1,000 ng/mL at 1 yr after the second HDCT/autoSCT. These patients had higher serum creatinine (0.62 vs 0.47 mg/mL, P = 0.007) than their counterparts (< 1,000 ng/mL). Post-SCT transfusion amount corresponded to increased ferritin levels at 1 yr after the second HDCT/autoSCT (P < 0.001). A lower CD34+ cell count was associated with a greater need of RBC transfusion, which in turn led to a higher serum ferritin level at 1 yr after HDCT/autoSCT. The number of CD34+ cells transplanted was an independent factor for ferritin levels at 1 yr after the second HDCT/autoSCT (P = 0.019). Consequently, CD34+ cells should be transplanted as many as possible to prevent the sustained iron overload after tandem HDCT/autoSCT and consequent adverse effects.