Iron Overload in Children With Leukemia; Identification of a Cutoff Value for Serum Ferritin Level.

OBJECTIVE: To determine the prevalence of iron overload in children with acute lymphoblastic leukemia (ALL) after treatment cessation and establish a cutoff value for serum ferritin level as an indicator of iron overload. BACKGROUND: Early detection and monitoring of iron overload in patients with leukemia is crucial. METHODS: In this prospective cohort study, 66 pediatric patients with ALL who were treated at a tertiary referral center affiliated with Shiraz University of Medical Sciences in Shiraz, Southern Iran, were investigated from July 2020 to December 2022. Serum ferritin levels were measured 6 months after treatment completion. T2* magnetic resonance imaging of the liver and heart was done for all patients. The receiver operating characteristic curve was used to illustrate the area under the receiver operating characteristic curve to assess the diagnostic value of serum ferritin level and total transfusion volume. RESULTS: A total of 24 patients (36.4%) had iron overload in the heart or liver based on T2 magnetic resonance imaging findings. Serum ferritin level was a highly accurate diagnostic marker for iron overload in pediatric patients with ALL, with a sensitivity of 95.8%, and specificity of 85.7% for a cutoff value of 238.5 ng/mL. Also, blood transfusion was a good predictor of iron overload a sensitivity of 75% and specificity of 81% for a cutoff value of 28.3 mL/kg. CONCLUSION: We identified specific cutoff values for serum ferritin and blood transfusion volume to predict iron overload with high sensitivity and specificity. These markers offer a cost-effective and accessible approach for periodic screening of iron deposition, particularly in resource-constrained settings.

Optical coherence tomography findings in patients with transfusion-dependent ß-thalassemia.

BACKGROUND: Structural ophthalmologic findings have been reported in patients with ß-thalassemia due to chronic anemia, iron overload, and iron chelation therapy toxicity in few previous studies. We aimed to investigate structural ocular findings and their relationship with hematological parameters in patients with transfusion-dependent ß-thalassemia (TDT). METHODS: In this cross-sectional study, from January 2018 to January 2019, 39 patients with TDT over the age of 18 participated. Multicolor fundus imaging, optical coherence tomography (OCT), and blue light fundus autofluorescence imaging were performed for all patients and 27 age- and sex-matched controls. RESULTS: The mean age of patients was 28.6 ± 6.2 years. The central macular thickness and macular thicknesses in all quadrants were significantly thinner in patients than controls (P<0.05). None of the retinal nerve fiber layer (RNFL) measurements were significantly different between TDT patients and controls. There was a significantly negative correlation between hemoglobin with central macula thickness (r=-0.439, P=0.005). All measurements of macular subfield thickness were insignificantly thinner in patients with diabetes mellitus (DM) compared to the non-DM subgroup. CONCLUSIONS: Macular thickness was significantly thinner in central macula and entire quadrants in TDT patients compared to healthy individuals; however, all RNFL measurement thicknesses were comparable between the two groups. Close monitoring of TDT patients by periodic ophthalmologic examinations with more focus on diabetic patients, patients with severe anemia and iron overload should be warranted.

Frequency of silent brain lesions and aspirin protection evaluation over 3 years follow-up in beta thalassemia patients.

Silent brain lesions might be associated with overt cerebrovascular accident over time in beta thalassemia major (BTM) and intermediate (BTI). Aspirin may be protective in these patients. We evaluated brain magnetic resonance imaging (MRI) in thalassemia patients to see whether aspirin is protective or not. A historical cohort study was conducted on 35 thalassemia patients, 22 BTI, and 13 BTM patients at Shiraz Hematology Research Center in 2018. Median age of the patients was 32 years and ranged from 8 to 42 years. Twenty-four patients (68.6%) were females. Overall frequency of white matter lesions (WMLs) in the first MRI was 10 patients (28.6%). After 3 years, 3 patients developed new lesions and the frequency of WMLs was 13 patients (37.1%) in the second MRI. Moreover, in 3 patients, number of WMLs increased. Patients with new lesions or more lesions compared to the baseline were significantly older than the other group (median age 36.5 years vs. 31 years, P = 0.046). Regarding aspirin consumption, only 1 patient (16.7%) of patients with new lesions was using aspirin compared to 10 (34.5%) of the other group (P = 0.640). The high-risk patients with thrombocytosis, splenectomy, severe iron overload, and older age (> 30 years) should be under close follow-up and evaluated on a regular periodic basis as well as brain MRI at least once every 3 years. Aspirin could be protective against new or progressive brain lesions so that low-dose aspirin is recommended in high-risk thalassemia patients.

Evaluation of the Effect of Support-Training System of Peer Group on Promotion of Self-Care in ß-Thalassemia Major Patients in Southern Iran.

ß-Thalassemia (ß-thal) is a chronic illness and its complications make the patient less compliant with the treatment protocol. This study aimed to evaluate the effect of a support-training system of peer group on promotion of self-care in ß-thal major (ß-TM) patients. In this semi-experimental study, 112 ß-TM were randomly selected and assigned to the study and control groups. Seven ß-TM patients, who were physically, mentally and socially normal, were selected and trained as the peer group. Eight training sessions over 4 months were done by the peer group. The questionnaire with 50 questions was scored using the Likert scale, always with 1 point, sometimes 0.5 point and never 0 points. In the study group, before intervention, the mean score of the patients was 29.84 ± 6.16, which, after intervention, increased significantly to 37.14 ± 4.35, p < 0.001. In the control group, the overall mean self-care patients' score decreased significantly, from 29.76 ± 7.18 to 29.48 ± 7.02. No significant difference was observed between pre and post intervention in the control group in all aspects of self-care (daily activities, fitness and wellness, nourishment, stress relief, job and home environment, time management, expression and creativity, support, items supporting self-care, self-care of the sickness), but it was significant in the study group. The results revealed the positive impact of peer group in promoting self-care of ß-TM patients in the study group compared to the control group, which may be used as a frontline educational model in these patients.

Evaluation of bone mineral density in patients with hemoglobin H disease.

OBJECTIVES: This study was conducted to assess bone mineral density (BMD) and bone mineral content (BMC) of patients with hemoglobin H (HbH) disease. METHODS: BMD and BMC were measured by dual energy X-ray absorptiometry of the lumbar spines and femur neck in 21 patients with Hb H disease over the age of 10 years. An association of BMD with sex, age, hemoglobin, calcium, phosphorus, and serum ferritin level was also evaluated. RESULTS: Prevalence of BMD below the expected range for age in the lumbar spine and femur neck region in patients with HbH disease were 33.3 and 14.3 %, respectively. Lumbar BMD was significantly lower in the patients compared to healthy individuals (median (min-max) 0.725 (0.595-0.924) vs. 1.061 (0.645-1.238), P < 0.001)). There was no significant relationship between BMD in the lumbar and femur neck with any of the evaluated variables (P value >0.05). CONCLUSION: Data regarding bone density in HbH disease is limited; osteoporosis as a common complication of ß-thalassemia intermedia syndrome should be considered even in HbH which shows its prevalence is less than ß-thalassemia intermedia.

Evaluation of the Relationship Between Hb F Levels and Nucleated Red Blood Cells with Morbidity in Non Transfusion-Dependent Thalassemia Patients.

Recognition of risk factors of morbidities in patients with ß-thalassemia intermedia (ß-TI) is an important issue that must be evaluated. Non transfusion-dependent thalassemia patients referred to the outpatient clinic of Shiraz University of Medical Science, Shiraz, South Iran were enrolled in this study between 2013 and 2014. Two peripheral blood smears were prepared for evaluating developmental stage of normoblasts. One hundred and thirty-one patients with ages ranging from 3 to 42 years (mean: 23.35 ± 7.9) were selected. Sixty-seven patients had at least one morbidity (51.1%). Osteoporosis and gallstones were the most common morbidities (33.6 and 24.4%, respectively). In the univariate model, hemoglobin (Hb), ferritin, Hb F, developmental stage of normoblasts and hydroxyurea (HU) therapy did not differ between patients with and without morbidity (p > 0.05) but mean age of patients and mean number of normoblasts were higher in patients with morbidity (p = 0.026 and p = 0.012, respectively). In the regression model, sex and splenectomy status were different between patients with and without morbidity. It seems that females and splenectomy are risk factors for morbidity in non transfusion-dependent thalassemia patients. [Sex: odds ratio (OR) = 2.21, 95% confidence interval (95% CI): 1.04-4.72, p = 0.39. Splenectomy: OR = 3.10, 95% CI: 1.12-8.59, p = 0.029.] This study shows that Hb F level and developmental stage of normoblasts does not effect the incidence of morbidities in non transfusion-dependent thalassemia patients but sex and splenectomy were effective factors in development of morbidities. Thus, splenectomy should be avoided as much as possible in patients with non transfusion-dependent thalassemia.

Family planning practices in families with children affected by ß-thalassemia major in Southern Iran.

Preventing the birth of children with ß-thalassemia major (ß-TM) is an important health issue. We investigated family planning practices and related factors among families with affected children. We selected a total of 569 parents from the parents of patients with ß-TM who were registered at thalassemia referral clinics in southern Iran. Information was recorded regarding demographic variables, socioeconomic status and family planning practices. The correlations between family planning practice and related factors were evaluated. Approximately 96.0% of the parents (546) were practicing contraception at the time of the study. Only 12.8% of the families whose first child had ß-TM decided to have no more children. The most frequent contraceptive method was tubal ligation (TL) (37.5%) followed by oral contraceptive pills (OCP) (31.5%). Higher education level of the mothers and higher economic status of the families were found to be related with the lower numbers of children with ß-TM (p = 0.001). We found a high percentage of safe contraception being used by at-risk couples. It seems that educational programs have been effective in influencing family planning practices. Further attention should be devoted to increasing the knowledge of at-risk couples with a greater focus on parents of low socioeconomic status. Because of cultural factors in Iran, many of these at-risk couples opted to achieve the desired family size, so implementation of a well-organized prenatal diagnostic system seems necessary.

The impact of inhibitors on the quality of life in patients with hemophilia.

Objective: To investigate the association of health-related quality of life in hemophilia patients with inhibitor and clinical and demographic characteristics. Methods: In this multi-center cross-sectional study, 41 male patients with hemophilia A were investigated from May to October 2021. All patients were registered at the Hemophilia Clinic affiliated with Shiraz and Zahedan Universities of Medical Sciences in Iran. Health-related quality of life of the patients was evaluated by the Short Form-36 questionnaire. Results: The patients' mean ± SD of age was 36.9 ± 13.2 (range: 18-76) years. Eleven patients (26.8%) were inhibitor positive. In univariate analysis, physical function, mental health dimension, and total Short Form-36 scores were significantly lower in the inhibitor-positive patients (p < 0.001, p = 0.045, and p = 0.035, respectively). Moreover, patients with severe disease showed significantly lower scores in physical function (p < 0.001), physical health dimension (p = 0.018), and total Short Form-36 (p = 0.031) than those with mild and moderate hemophilia. Also, blood-borne infections showed a significant association with lower score in physical health dimension (p = 0.038). In addition, annual bleeding rate showed significant negative correlations with physical health dimension (rs = -0.609, p < 0.001), mental health dimension (r = -0.317, p = 0.044), and total Short Form-36 (r = -0.455, p = 0.003) scores. In multiple linear regression analysis, disease severity revealed a significant negative relationship with scores in physical function (p = 0.001), role physical (RP) (p = 0.015), general health (GH) (p = 0.006), physical health dimension (p = 0.006), and marginally in total Short Form-36 score (p = 0.054). Also, age of the patients showed a significant negative association with physical function and GH scores (p < 0.001 and p = 0.015, respectively). Conclusion: Disease severity and age were shown as independent factors affecting health-related quality of life, but inhibitor alone was not an independent influencing factor. Reduced health-related quality of life was also observed in hemophilia patients with higher annual bleeding rate and blood-borne infections. Therefore, it is necessary to pay more attention to these subgroups. Further studies with larger sample size are needed for more accurate results.

Trace Elements in Children with Acute Lymphoblastic Leukemia.

OBJECTIVE: Although combined chemotherapy regimen leads to 80% remission in children with acute lymphocytic leukemia (ALL), malnutrition and altered serum trace elements as a consequence of chemotherapy agents, have become the new issue to deal with. With the aim to evaluate each trace element in childhood ALL, we investiguâtes six main trace elements before and after induction chemotherapy while considering age, gender and chemotherapy protocol as confounding factors. METHODS: Thirty-six newly diagnosed ALL children were recruited, and trace elements were assessed by atomic absorption spectrometry technique. Trace elements (Zinc, Copper, Manganese, Magnesium, Chromium and Iron) decreased significantly after induction chemotherapy. RESULTS: Considering the confounding factors, mean difference of elements decreased significantly, except for Chromium. Its mean difference was only significant in children younger than 10 and those who had received standard risk chemotherapy. CONCLUSION: In conclusion, all the studied trace elements decreased significantly after induction chemotherapy session in ALL children. This highlights the importance of complementary and supplementary management. A larger cohort study with longer follow up is warranted to elucidate the long-term effect of chemotherapy on these trace elements on the general health status, quality of life or risk of relapse in ALL children.

Epidemiologic study of patients with thrombotic events referred to a tertiary hospital in Southern Iran.

BACKGROUND AND AIM: Thromboembolic events mainly occur in older age is related with high morbidity and mortality, and considerable health-care costs particularly in developing countries. Both arterial and venous thromboembolism has known risk factors such as hyperlipidemia, obesity, diabetes, cancer, major surgery, central catheter. We aimed to evaluate the occurrence of thrombotic events and related risk factors in a group of Iranian patients. METHODS: In this cross-sectional study, all patients (n = 99) who were complicated by thrombotic events referred to the Hematology Research Center of Shiraz University of Medical Sciences were investigated from 2015 to 2017, in Shiraz, Southern Iran. Data were collected from their medical records by a designed data gathering form. RESULTS: The median age of the occurrence of thrombosis was 51 (IQR: 31) years. From all thrombotic events 52.5% occurred in females. Venous thrombosis was more prevalent than arterial (61.6% vs. 38.4%). Hypertension, diabetes mellitus and ischemic heart disease were the most associated disease with thrombosis. Most of the patients (79.8%) had no episodes of relapse and the occurrence of relapse had no significant relationship with thrombophilia and underlying disease. Acceptable response rate for warfarin therapy was achieved in 46.5% with 5 mg and 43.4% with 5-7.5 mg. CONCLUSION: Knowing the frequency and risk factors for thrombotic events lead to timely diagnosis and management of thrombosis. Atrial fibrillation and valvular rheumatic heart disease are the most common risk factors of thrombosis in our study showing prophylaxis is necessary in high-risk patients.

Breastfeeding as a Protective Effect Against Childhood Leukemia and Lymphoma.

BACKGROUND: Over the past several years, breastfeeding has been associated with many benefits as well as protective effects against many diseases. There is limited evidence for the relationship between breastfeeding and the incidence of leukemia. OBJECTIVES: In this study, we evaluate the correlation of childhood leukemia and lymphoma with breastfeeding duration in children in southern Iran. PATIENTS AND METHODS: Through this case control study, we compared 123 patients with leukemia and lymphoma to a control group of 137 healthy children. Statistical analysis was done using the Chi-square test and t-test as well as logistic regression methods. A P-value of less than 0.05 was considered significant. RESULTS: Our findings showed that breastfeeding duration had no significant difference between cases and controls. However, the rural living percentage in patients with leukemia and lymphoma was higher than in the control group (39.8% versus 14.6% [P < 0.001 and OR = 3.87]) and parents' exposure to chemical materials during the war between Iran and Iraq was higher in sick patients (6.5% versus 0% [OR = 20.2%]). CONCLUSIONS: The current study showed that breastfeeding duration has no protective effect against childhood leukemia and lymphoma. In addition, we suggest that some factors such as living in a rural area, smoking during pregnancy, parents' exposure to chemical materials and low socioeconomic status can increase the incidence rate of childhood leukemia and lymphoma.

Quality of life among Iranian patients with beta-thalassemia major using the SF-36 questionnaire.

CONTEXT AND OBJECTIVE Patients with beta-thalassemia major (ß-TM) experience physical, psychological and social problems that lead to decreased quality of life (QoL). The aim here was to measure health-related QoL and its determinants among patients with ß-TM, using the Short Form-36 (SF-36) questionnaire. DESIGN AND SETTING Cross-sectional study at the Hematology Research Center of Shiraz University of Medical Sciences, in southern Iran. METHODS One hundred and one patients with ß-TM were randomly selected. After the participants' demographics and disease characteristics had been recorded, they were asked to fill out the SF-36 questionnaire. The correlations of clinical and demographic factors with the QoL score were evaluated. RESULTS There were 44 men and 57 women of mean age 19.52 ± 4.3 years (range 12-38). On two scales, pain (P = 0.041) and emotional role (P = 0.009), the women showed significantly lower scores than the men. Lower income, poor compliance with iron-chelating therapy and presence of comorbidities were significantly correlated with lower SF-36 scores. These factors were also found to be determinants of worse SF-36 scores in multivariate analysis. CONCLUSIONS We showed that the presence of disease complications, poor compliance with iron-chelating therapy and poor economic status were predictors of worse QoL among patients with ß-TM. Prevention and proper management of disease-related complications, increased knowledge among patients regarding the importance of managing comorbidities and greater compliance with iron-chelating therapy, along with psychosocial and financial support, could help these patients to cope better with this chronic disease state.

Quality of life among Iranian patients with beta-thalassemia major using the SF-36 questionnaire / Qualidade de vida em pacientes iranianos com beta-talassemia maior usando o questionario SF-36

CONTEXT AND OBJECTIVE Patients with beta-thalassemia major (β-TM) experience physical, psychological and social problems that lead to decreased quality of life (QoL). The aim here was to measure health-related QoL and its determinants among patients with β-TM, using the Short Form-36 (SF-36) questionnaire. DESIGN AND SETTING Cross-sectional study at the Hematology Research Center of Shiraz University of Medical Sciences, in southern Iran. METHODS One hundred and one patients with β-TM were randomly selected. After the participants' demographics and disease characteristics had been recorded, they were asked to fill out the SF-36 questionnaire. The correlations of clinical and demographic factors with the QoL score were evaluated. RESULTS There were 44 men and 57 women of mean age 19.52 ± 4.3 years (range 12-38). On two scales, pain (P = 0.041) and emotional role (P = 0.009), the women showed significantly lower scores than the men. Lower income, poor compliance with iron-chelating therapy and presence of comorbidities were significantly correlated with lower SF-36 scores. These factors were also found to be determinants of worse SF-36 scores in multivariate analysis. CONCLUSIONS We showed that the presence of disease complications, poor compliance with iron-chelating therapy and poor economic status were predictors of worse QoL among patients with β-TM. Prevention and proper management of disease-related complications, increased knowledge among patients regarding the importance of managing comorbidities and greater compliance with iron-chelating therapy, along with psychosocial and financial support, could help these patients to cope better with this chronic disease state. .

CONTEXTO E OBJETIVO Pacientes com beta-talassemia maior (β-TM) vivenciam problemas físicos, psicológicos e sociais que levam à diminuição da qualidade de vida (QV). O objetivo foi determinar a QV relacionada à saúde e seus determinantes em pacientes com β-TM, utilizando questionário SF-36 (Short Form-36). TIPO DE ESTUDO E LOCAL Estudo transversal no Centro de Hematologia e Pesquisa em Universidade de Ciências Médicas de Shiraz, no sul do Irã. MÉTODOS Foram selecionados aleatoriamente 101 pacientes com β-TM. Após registro demográfico e características da doença, eles foram convidados a preencher o questionário SF-36. A correlação entre fatores clínicos e demográficos com escore de QV foi avaliada. RESULTADOS Havia 44 homens e 57 mulheres, com idade média de 19,52 ± 4,3 (variação 12-38) anos. Em duas escalas, dor (P = 0,041) e aspectos emocionais (P = 0,009), as mulheres apresentaram escores significativamente menores aos dos homens. Menor renda, baixa adesão à terapia quelante de ferro e presença de comorbidades foram correlacionadas com escores SF-36 significativamente menores. Esses fatores foram também considerados determinantes de piores escores de SF-36 em análise multivariada. CONCLUSÕES Mostramos que a presença de complicações da doença, a baixa adesão ao tratamento da terapia quelante de ferro e o baixo status econômico são preditores de pior QV em pacientes com β-TM. Prevenção e manejo adequado das complicações relacionadas com a doença, aumento do conhecimento dos pacientes sobre a importância do gerenciamento de comorbidades e ter maior adesão ao tratamento quelante de ferro, considerando também ...