BACKGROUND:CD27 deficiency is a rare primary immunodeficiency associated with severe Epstein-Barr virus (EBV)-related complications, including lymphoma. OBSERVATIONS:We report a pediatric patient with CD27 deficiency who developed EBV-associated diffuse large B-cell lymphoma with primary CNS involvement and early relapse. The patient underwent unmanipulated haploidentical stem cell transplantation using post-transplant cyclophosphamide. Engraftment was successful, graft-versus-host disease was controlled, and full donor chimerism was achieved. CONCLUSIONS:After 40 months, the patient remains free of GVHD, PID, and lymphoma relapse, demonstrating successful lymphoma control and correction of the underlying immunodeficiency, thereby highlighting the feasibility of haploidentical HSCT in these patients.

Background: Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy. Despite major therapeutic advancements, relapse and treatment failure continue to be serious challenges, particularly in low- and middle-income countries. While several well-established prognostic factors such as age, white blood cell (WBC) count, cytogenetic abnormalities, minimal residual disease (MRD), and socioeconomic status (SES) have been validated in high-income regions, limited evidence is available from developing countries. Therefore, this study aimed to investigate the prognostic factors associated with relapse and mortality in children diagnosed with ALL in a developing country setting. Materials and Methods: This prospective cohort study included 130 children younger than 15 years with confirmed ALL admitted to a tertiary referral center from 2014 to 2020. Demographic, clinical, laboratory, MRD, genetic, and socioeconomic data were collected from the patients, and they were followed for up to 48 months after achieving remission. Univariate and multivariate Cox regression models were used to identify the independent predictors of relapse and mortality (significance threshold: p < 0.05). Results: The mean age of the participants was 4.76 ± 3.36 years, and relapse or death occurred in 31 (23.8%) of them. The univariate analysis showed that older age, MRD > 0.1% on day 33, and higher hemoglobin level were associated with relapse. The multivariate analysis identified MRD > 0.1% on day 33 as the only significant independent predictor of relapse. A survival analysis via a multivariate Cox-regression model also showed a lower income level (HR = 2.150, p = 0.033) associated with a higher mortality. Conclusion: This study reveals MRD on day 33 as the strongest determinant of relapse and highlights the critical role of socioeconomic disparities in mortality among children with ALL in developing countries. Early MRD-based risk stratification and policies aimed at bridging socioeconomic gaps may contribute to improved survival outcomes. Larger multicenter studies and extended molecular profiling are recommended.

Acute leukemia is the most commonly diagnosed malignancy in children. In addition to genetic factors, exposure to chemicals also increases its risk. Considering the increasing incidence of pediatric acute leukemia and limited experience on the underlying role of environmental pollutants, this study aims to assess the relationship between exposure to benzene and organochlorine compounds and pediatric acute leukemia. Participants of this cross-sectional study were children aged under 15 years diagnosed as new cases of acute leukemia and healthy children. Urine samples were collected for measurement of benzene and organochlorine pesticides metabolites by using liquid-liquid microextraction techniques. This study comprised 496 participants including 195 (39.31

Eosin Yellowish (Eosin-Y) is an effective treatment option for a range of dermatological conditions. It is crucial to carefully identify patients presenting systemic adverse reactions related to topical aqueous Eosin-Y overuse and to monitor them closely during short-term use to prevent severe complications.

A 19-month-old female with Down syndrome presented with recurrent left cheek swelling and left eye involvement, initially diagnosed as fasciitis/myositis and periorbital cellulitis. Despite empiric antibiotics, symptoms persisted. A whole-body CT scan revealed prominent lymph nodes, and a biopsy of the buccal mass showed myeloid sarcoma. Bone marrow aspiration confirmed acute myeloid leukemia. Following chemotherapy, the patient’s symptoms resolved. This case underscores that acute leukemia may manifest as facial swelling or periorbital cellulitis, highlighting the importance of considering extramedullary myelosarcoma in cases of persistent, unexplained soft tissue swelling that does not respond to standard treatments.

Vitamin D deficiency/insufficiency (VDD, VDI) is common in children yet limited experience exists on the association of VDD and hematologic malignancies amongst this population. Therefore, this study aimed to compare serum vitamin D levels in children with acute lymphoblastic leukemia (ALL) and controls. Moreover, vitamin D levels is compared in subjects with and without relapse and evaluated as a prognostic factor for relapse-free survival (RFS). Children with newly diagnosed ALL were recruited as case group. Data on demographic variables as well as the dietary habits were collected by interview. In addition, serum 25(OH)D3 was measured. The case group was followed up for 36 months to assess RFS. Overall, 358 subjects were included in the study (n = 169 cases, n = 189 controls). The mean levels of 25(OH)D3 were 28.05 & PLUSMN; 18.87 and 28.76 & PLUSMN; 12.99 in cases and controls, respectively (p = .68). VDD was found in 15.4% (n = 26) and 4.2% (n = 8) of the case and control groups, respectively (p < .001). Relapse was seen in 18.34% of patients and vitamin D levels of 20 ng/mL or above were associated with longer RFS (p = .044 by log-rank test). In this study, VDD and VDI amongst children with ALL were significantly higher than controls. In addition, lower levels of Vitamin D were associated with increased risk of relapse.

BackgroundRecent studies have demonstrated hematogones (HGs) expansion to be associated with favorable outcomes in hematological diseases, especially in patients with acute myeloid leukemia and patients undergoing hematopoietic stem cell transplantation. Acute lymphoblastic leukemia (ALL) is the most common form of cancer in children. As of now, minimal residual disease (MRD) remains the most compelling independent prognostic factor in childhood ALL. There is need for more prognostic tools for evaluating relapse risk. ProcedureThe goal of this study was to assess the prognostic value of HGs on relapse-free survival (RFS) and overall survival (OS) in childhood ALL. In this prospective cohort study, a total of 122 subjects with definitive diagnosis of precursor B lymphoblastic leukemia were evaluated. Flow cytometric HG detection was performed in bone marrow aspirates after induction and consolidation therapy. ResultsThe median follow-up period of patients was 35.5 +/- 9.4 (SD) months. Patients who had at least 1.0% HGs had a significantly better RFS (p = .023). Moreover, univariate and multivariate analyses confirmed that positive HGs were independently associated with longer RFS (unadjusted model: hazard ratio = 0.33, 95% CI = 0.12-0.91, p = .031; adjusted model: hazard ratio = 0.30, 95% CI = 0.11-0.82, p = .020). ConclusionsAlong with the role of MRD, our study shows the significance of HGs as an independent prognostic factor. The results indicate the independent prognostic value of HGs on RFS after adjustment for other prognostic factors, and can be beneficial for risk stratification and treatment modifications amongst pediatric B-cell ALL patients.

Background:Acute leukemia is the most common type of malignancy in children, and no major environmental risk factors have been identified relating to its pathogenesis. This study has been conducted with the aim for identifying risk factors associated with this disease. Methods:This study was conducted in 2016-2020 among children aged <15 years residing in Isfahan Province, Iran. Children with newly diagnosed Acute lymphoblastic leukemia, including Acute myeloid leukemia (ALL and AML) were considered a case group. The control group was selected among children hospitalized in orthopedic and surgery wards in the same region. Demographic data, parental occupational exposures and educational level, maternal obstetric history, type of feeding during infancy and parental smoking habits, exposure to pesticides, and hydrocarbons besides dietary habits (using a food frequency questionnaire) were evaluated. Results:Overall, 497 children (195 cases and 302 controls) completed the survey. In the initial analysis, there was no significant difference between case and control groups about type of milk feeding (P = 0.34) or parental age (P = 0.56); however, an association between mothers' education and increased risk for ALL was observed (P = 0.02). Conclusions:The results of this study can be helpful in better understanding the environmental risk factors involved in the incidence of acute leukemia. Future publications based on the analysis of the database created in the present study can lead to recognizing these factors. In addition, evaluating the effect of these factors on treatment outcomes is an important step in reducing the burden of the disease.

Background: Beta-thalassemia major is a type of inherited blood disease that results in variable outcomes such as severe anemia due to haemoglobin chains. Recurrent and lifelong blood transfusions as a treatment in beta-thalassemia major disease lead to iron deposition in various organs and cause the failure of multiple organs. Failure of affected organs leads to Body mass index (BMI) abnormality. This study aimed to evaluate the association between BMI and serum ferritin level as a marker for iron overload. Materials and Methods: A cross-sectional study designed and conducted with total number of 740 paediatrics, with mean age about 14.2±8.7 years old and with beta-thalassemia major requiring recurrent blood transfusion. Patient information, including demographics, serum ferritin level and percentage of BMI, was recorded and analysed by SPSS 25.0 and the statistical significant level, considered as 0.05. Results: A total number of 740 paediatrics with beta-thalassemia major disease (mean age about 14.2±8.7 years) were included to study to examine the association between serum ferritin level and their BMI. The total mean serum level of ferritin calculated about 3326 ± 3859 Nanogram/mililitter (ng/ml). Totally, 447 (60.4%) case of them had BMI percentile less than 5%, 274 (37.02%), 16 (2.16%) and 3 (0.4%) had BMI percentile 5%-85%, 85%-95% and more than 95%. There was no relation between gender and serum ferritin levels. The relationship between age and BMI has been positive (P=0.002). Finally, it resulted that there was a negative relationship between the BMI percentile and mean serum ferritin levels in paediatrics with beta-thalassemia major (P=0.031). Conclusion: Frequent Blood transfusion is associated with elevated serum ferritin level in paediatrics with beta-thalassemia major disease and experiencing lower percentiles of BMI in these patients.

Objective. We compared the glucose-6-phosphate dehydrogenase (G6PD) deficiency screening costs in neonates and treatment costs for patients with favism who were referred to the government hospitals of Isfahan with acute hemolysis. Methods. In this retrospective study, 116 patients with favism were selected, and their clinical data and treatment costs were recorded in Iranian Rials (IRR) and United States Dollars (USD). The costs of neonatal G6PD screening tests were estimated based on quantitative and qualitative screening kits by referring to each hospital's laboratories. Approximately 84760 births occurred in Isfahan province from October 2014 to October 2015. Results. Based on our results, 73 patients (62.9%) were males, and 43 of them (37.1%) were females. The causes for hemolysis were the consumption of broad beans in 78.4%, infection in 19.8%, and drugs (erythromycin and sodium cephalothin) in 1.7% of patients. The majority of patients (46.6%) received a single blood transfusion, and 18.9% received two or more transfusions, while 34.5% had received no blood transfusions. The mean and standard deviation of patients' medical expenses were 7,472,005±7,329,847 IRR (276.74±271.47 USD). The total cost of treatment for patients was 869,162,324 IRR (32,191.19 USD). The screening cost for all newborns from October 2014 to October 2015 with qualitative kits was 754,364,000 IRR (27,939.40 USD) and with the quantitative kits was 960,330,800 IRR (35,567.80 USD). Conclusion. The costs of the qualitative screening test for G6PD deficiency are lower than the costs of treatments of favism patients in Isfahan.

Background: Malignant disorder with B or T stem cell basis leads to development and continuation of acute lymphoblastic leukemia (ALL) due to aggregation of blast cells in bone marrow. The environmental, genetic, and demographic factors may influence the disease relapse. The objective of this study was to assess the relation between end of induction minimal residual disease and different risk factors in patients with ALL. Materials and Methods: This analytic-descriptive study consisted of 91 patients with ALL who referred to Seyed Alshohada Hospital, Isfahan, Iran. The mean age of the patients was 4.91 3.07 years old. The patients were assessed in terms of demographic characteristics, socioeconomic status, and treatment protocol. Their treatment began with Prednisolon, Dexamethason, Vincristine, L-Asparginase (L.APS) or (PEG-ASP), and Anthracycline for 28 days. Then, the end of induction minimal residual disease was assessed in each patient. For data analysis, Spierman, Mann Whitney, and Kruskal wallis tests were applied. Results: The monthly income level of the patients' families were poor, and we found a significant correlation between monthly income level of the patients' families and the incidence of minimal residual disease (P=0.03). None of the studied factors, including age, the mean of white blood cell count in the first complete blood count, hemoglobin level, platelet level, gender, central nervous system, mediastinal mass, splenomegaly, hepatomegaly, translocation, parents' education, and parents' occupation and response to corticosteroid treatment that might have had not any impacts on the studied disease(p>0.05). Conclusion: In this study, it was found that assessing the effect of risk factors on the minimal residual disease in patients with leukemia could be a good solution for detecting and eliminating risk factors and increasing the relapse time.

No previous studies were found to examine the effect of soy as a whole food on patients with leukemia. The present randomized controlled clinical trial studied the effect of soy nut on children with B-cell acute lymphoblastic leukemia (ALL) who were in the maintenance phase of chemotherapy. The eligible patients were randomized to receive 30g/day soy or cowpea nut powder for 12weeks. Dietary intake, physical activity, anthropometric measurements, complete blood count, serum albumin, serum highly sensitive C-reactive protein (hs-CRP), and Tumor necrosis factor alpha (TNF-) as well as chemotherapy side effects were assessed at the start and the end of the study. In total 29 and 27 children completed the study (aged 6.34 +/- 2.44 and 5.85 +/- 2.35years) in soy and cowpea nut groups, respectively. The total energy and protein intake, and physical activity as well as body weight, body mass index, number of red blood cells, hemoglobin and hematocrit levels, and fatigue were significantly improved in the soy nut group compared to patients who consumed cowpea nut (P<0.05). Soy nut intake might improve the nutritional status, anemia, and fatigue in children with ALL. Studies targeting blood cell fractions and disease recurrence are highly recommended.

Background: Cardiac complications due to iron overload are the most common cause of death in patients with thalassemia major. The aim of this study was to compare iron chelation effects of deferoxamine, deferasirox, and combination of deferoxamine and deferiprone on cardiac and liver iron load measured by T2* MRI. Methods: In this study, 108 patients with thalassemia major aged over 10 years who had iron overload in cardiac T2* MRI were studied in terms of iron chelators efficacy on the reduction of myocardial siderosis. The first group received deferoxamine, the second group only deferasirox, and the third group, a combination of deferoxamine and deferiprone. Myocardial iron was measured at baseline and 12 months later through T2* MRI technique. Results: The three groups were similar in terms of age, gender, ferritin level, and mean myocardial T2* at baseline. In the deferoxamine group, myocardial T2* was increased from 12.0±4.1 ms at baseline to 13.5±8.4 ms at 12 months (p=0.10). Significant improvement was observed in myocardial T2* of the deferasirox group (p<0.001). In the combined treatment group, myocardial T2* was significantly increased (p<0.001). These differences among the three groups were not significant at the 12 months. A significant improvement was observed in liver T2* at 12 months compared to baseline in the deferasirox and the combination group. Conclusion: In comparison to deferoxamine monotherapy, combination therapy and deferasirox monotherapy have a significant impact on reducing iron overload and improvement of myocardial and liver T2* MRI.

Background: There are several medications for treatment of immune thrombocytopenic purpura (ITP), including corticosteroids, intravenous immunoglobulin, immunosuppressive drugs, and even splenectomy. In case of failure, Rituximab as one of the medications used in these patients should be considered. Method: This Case-series study was conducted prospectively in patients who were referred to Hematology & Oncology Clinic of Ali Asghar Hospital. Eighteen Patients were followed up for at least 3-5 years. Results: Four weeks after treatment, only in 6 patients (33/3%) of 18 patients, complete response (Plt > 100,000) were obtained and most patients (67/66%) had no appropriate response. In subsequent surveys conducted at 6 and 36 months after treatment, the percentage of patients responding fell to 22/2% hit. Conclusion: The results of this study demonstrate proper safety of Rituximab in the treatment of chronic ITP. However, the drug had no significant effect on the expected improvement in platelet count of patients. It seems that monotherapy is ineffective in the treatment of chronic ITP and combination with other complementary therapies is recommended.

Rhabdomyosarcomas are the most common soft tissue sarcoma in adult and children that accompany with skeletal muscle differentiation. Skin metastasis of rhabdomyosarcomas is unusual and has only been sporadically reported in literature. In this paper we present a case of skin metastasis of rhabdomyosarcoma in an 8-year-old girl that has treated with chemotherapy.

The patient was a 1.5-year-old boy who presented with growth failure, inability to walk, polyuria, and polydipsia.On physical examination, he had fair skin and blond hair.He did not have organomegaly.Ophthalmologic examination by an ophthalmologist was normal.Hypophosphatemia, hypovitaminosis D, mild acidosis, glucosuria, proteinuria, and generalized aminoaciduria were found upon laboratory studies.He underwent bone marrow aspiration that revealed increased numbers of macrophages containing polygonal crystals.

Leishmaniasis is a parasitic disease and often seen in developing countries and tropic areas. Visceral leishmaniasis (VL) is the most severe form of this disease, which is fatal if left untreated. In this report, we describe an 11-month-old infant with poor growth, pancytopenia, and splenomegaly. Microscopic examination of bone marrow revealed intracellular and extracellular Leishmania amastigotes. VL should be considered when a child presents with fever, failure to thrive, organomegaly, and pancytopenia.

Arti” cial neural networks are intelligent systems that have been successfully used for prediction in different medical ” elds. In this study, ef” ciency of neural networks for prediction of lupus nephritis in patients with systemic lupus erythematosus (SLE) was compared with a logistic regression model and clinicians’diagnosis. Overall accuracy, sensitivity and speci” city of the optimal neural network were 68.69, 73.77 and 62.96%, respectively. Overall accuracy of neural network was greater than the other two methods (P-value< 0.05). The neural network was more speci” c in predicting lupus nephritis (P-value < 0.01), but there was no signi” cant difference between sensitivities of the three methods. Sensitivities of all three methods were greater than their speci” cities. We concluded that neural networks are ef” cient in predicting lupus nephritis in SLE patients.