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We studied the alpha-globin gene abnormalities, the clinical features, hematologic values, growth assessment, transfusion therapy, and serum ferritin levels of patients with hemoglobin H (HbH) disease in southern Thailand. HbH disease in 83 of the 147 patients was the deletional type of HbH. The remaining 64 patients was the nondeletional type of HbH disease. All 83 patients with the deletional type were double heterozygotes of alpha(0)-thalassemia and alpha(+)-thalassemia. The Southeast Asian type of alpha(0)-thalassemia accounted for 98% of the Thai patients with HbH disease and the Thai type of alpha(0)-thalassemia made up the rest. A 3.7-kb deletion accounted for 91% of alpha(+)-thalassemia, and a 4.2-kb deletion made up the rest of the deletional type. In patients with nondeletional type of HbH disease, the Constant Spring variant was the majority of the disease. Newborns with a nondeletional genotype had higher mean corpuscular volume, had higher mean corpuscular hemoglobin, had higher red blood cell distribution width, had lower mean corpuscular hemoglobin concentration, and had higher proportions of Hb Bart's than those with a deletional genotype. Twenty-one percent of children with HbH disease had growth deficiency. A genotype-phenotype correlation was found; patients with the nondeletional type of HbH disease had more symptoms at a younger age, more severe hemolytic anemia, more growth deficiency, more dysmorphic facial features, larger spleens, larger livers, and higher serum ferritin levels and required more transfusions than patients with deletional HbH disease.

Background Cardiac and liver iron assessment using magnetic resonance imaging (MRI) is non-invasive and used as a preclinical "endpoint" in asymptomatic patients and for serial iron measurements in iron-overloaded patients. Purpose To compare iron measurements between hepatic and myocardial T2* and T2 at 1.5T and 3T MRI in normal and iron-overloaded patients. Material and Methods The T2 and T2* values from the regions of interest (ROIs) at mid-left ventricle and mid-hepatic slices were evaluated by 1.5T and 3T MRI scans for healthy and iron-overloaded patients. Results For iron-overloaded patients, the myocardial T2 (1.5T) and myocardial T2 (3T) values were 60.3 ms (range = 56.2-64.8 ms) and 55 ms (range = 51.6-60.1 ms) (ρ = 0.3679) while the myocardial T2* (3T) 20.5 ms (range = 18.4-25.9 ms) was shorter than the myocardial T2* (1.5T) 35.9 ms (range = 31.4-39.5 ms) (ρ = 0.6454). The hepatic T2 at 1.5T and 3T were 19.1 ms (range = 14.8-27.9 ms) and 15.5 ms (14.6-20.4 ms) (ρ = 0.9444) and the hepatic T2* at 1.5T and 3T were 2.7 ms (range = 1.8-5.6 ms) and 1.8 ms (range = 1.1-2.9 ms) (ρ = 0.9826). The line of best fit exhibiting the linearity of the hepatic T2* (1.5T) and hepatic T2* (3T) had a slope of 2 and an intercept of -0.387 ms (R = 0.984). Conclusion Our study found myocardial T2 (1.5T) nearly equal to T2 (3T) with myocardial T2* (3T) 1.75 shorter than myocardial T2* (1.5T). The relationship of hepatic T2* (1.5T) and hepatic T2* (3T) was linear with T2* (1.5T) approximately double to T2* (3T) in iron-overloaded patients. This linear relationship between hepatic T2* (1.5T) and hepatic T2 (3T) could be an alternative method for estimating liver iron concentration (LIC) from 3T.

Both childhood cancer incidence and survival rates have increased, suggesting improvement in the health care system as more cases are identified and treated. Analyzing childhood cancer trends in low- and middle-income countries can improve understanding of cancer etiology and pediatric health care disparities.

NK cells play an important role in hematopoietic stem cell transplantation (HCT) and in cross talk with dendritic cells (DCs) to induce primary T cell response against infection. Therefore, we hypothesized that blood DCs should augment NK cell function and reduce the risk of leukemia relapse after HCT. To test this hypothesis, we conducted laboratory and clinical studies in parallel. We found that although phenotypically NK cells could induce DC maturation and DCs could in turn increase activating marker expression on NK cells, paradoxically, both BDCA1+ myeloid DCs and BDCA4+ plasmacytoid DCs suppressed the function of NK cells. Patients who received an HLA-haploidentical graft containing larger number of BDCA1+ DCs or BDCA4+ DCs had a higher risk of leukemia relapse and poorer survival. Further experiments indicated that the potent inhibition on NK cell cytokine production and cytotoxicity was mediated in part through the secretion of IL-10 by BDCA1+ DCs and IL-6 by BDCA4+ DCs. These results have significant implications for future HCT strategies.

Background Prognosis of low-grade glioma are currently determined by genetic markers that are limited in some countries. This study aimed to use clinical parameters to develop a nomogram to predict survival of patients with diffuse astrocytoma (DA) which is the most common type of low-grade glioma. Materials and Methods Retrospective data of adult patients with DA from three university hospitals in Thailand were analyzed. Collected data included clinical characteristics, neuroimaging findings, treatment, and outcomes. Cox's regression analyses were performed to determine associated factors. Significant associated factors from the Cox regression model were subsequently used to develop a nomogram for survival prediction. Performance of the nomogram was then tested for its accuracy. Results There were 64 patients with DA with a median age of 39.5 (interquartile range [IQR] = 20.2) years. Mean follow-up time of patients was 42 months (standard deviation [SD] = 34.3). After adjusted for three significant factors associated with survival were age ≥60 years (hazard ratio [HR] = 5.8; 95% confidence interval [CI]: 2.09-15.91), motor response score of Glasgow coma scale < 6 (HR = 75.5; 95% CI: 4.15-1,369.4), and biopsy (HR = 0.45; 95% CI: 0.21-0.92). To predict 1-year mortality, sensitivity, specificity, positive predictive value, negative predictive value, accuracy, and area under the curve our nomogram was 1.0, 0.50, 0.45, 1.0, 0.64, and 0.75, respectively. Conclusions This study provided a nomogram predicting prognosis of DA. The nomogram showed an acceptable performance for predicting 1-year mortality. AbstractKeywords ► diffuse astrocytoma ► nomogram ► survival analysis

Iron overload has not been studied extensively and prospectively in pediatric survivors of allogeneic hematopoietic stem cell transplantation (HSCT); therefore, we conducted a prospective long-term study of 133 survivors of childhood leukemia to assess the incidence of and risk factors for iron overload and to investigate its association with organ dysfunction. One yr after HSCT, the mean serum ferritin level was 1158 ng/mL (range, 22-3264 ng/mL), with 124 patients (93.2%) having a serum ferritin level that exceeded the upper limit of the normal range (110 ng/mL). Thereafter, the serum ferritin level declined over time. There was a positive correlation between the level of serum ferritin and that of total bilirubin (r = 0.21, p < 0.001) and glutamate pyruvate transaminase (r = 0.17, p < 0.001). A high concentration of serum ferritin was associated with low cardiac fractional shortening (r = -0.15, p = 0.047). In addition, patients with hypothyroidism and GH deficiency had a higher level of serum ferritin than those without (p < 0.02). We conclude that iron overload is common after HSCT and is associated with hepatic, cardiac, and endocrine dysfunction.

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