Alok Srivastava

..These results demonstrate that dapsone is an effective, inexpensive and well-tolerated treatment for chronic ITP, in both children and adults and could be considered for patients who fail steroid therapy...

..Efforts are needed to develop national registries and make at least basic services for diagnosis and treatment widely available...

..Additionally, this regimen has minimal toxicity and can be administered on an outpatient basis after remission induction...

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..Until such time as these examples are widely emulated, less safe products will continue to be used in situations where a distinction frequent needs to be made between what is ideal and what is practical...

..Furthermore, these tests need to be standardized with regard to their reagents, methodology and interpretation, and finally, much more data need to be collected regarding clinical correlations with the parameters measured...

..The combined experience in the developing world in providing hemophilia services should be used to define standards of care that are practical and to set achievable goals...

..Less intensive protocols could achieve similar outcomes. Large multi-centre prospective studies are needed to provide comparative data on unresolved issues so that factor replacement therapy can be optimized, based on evidence...

..001, respectively). We conclude that the GSTM1-null genotype predisposes to HVOD, and the sinusoidal endothelial cells and hepatocyte damage may be mediated by metabolites of busulfan through depletion of the cellular GSH pool...

..Platelet transfusions are often needed to control the bleeding. Glanzmann thrombasthenia results from mutations in the genes located on chromosome 17q21-23, encoding the platelet glycoprotein (GP) IIb/IIIa receptor...

..This can be achieved by production of such concentrates locally or by importing them. Different models are possible depending on the circumstances in each country...

..There is inadequate awareness of this condition and lack of support for these patients from the health care system in many developing countries. Concerted efforts are needed at the scientific and social levels to improve this situation...

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..There is potential for pretransplant manipulation of this cellular subset in the graft...

..At a median follow up of 30 months, 11 patients (61.1%) were alive and well. Fludarabine-based conditioning regimens with PBSC transplantation can be used successfully in patients with aplastic anemia and fungal infections...

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..On a multivariate analysis, patients with a day 28 NK cell count below the median value of 142/microL had a significantly higher rejection rate (hazard ratio [HR] = 11.1, P = .038) and a lower EFS (HR = 16.3, P = .034)...

..Results in the low-risk group are comparable to that reported with conventional therapy while additional interventions would probably be required in high-risk cases...

..In a first paper from India, we describe our experience in establishing a flow cytometry-based MRD assay for precursor B lineage ALL (BCP-ALL) with emphasis on the assay standardization and cost...

..2%, respectively. High-risk group patients and those that remain RT-PCR positive at the end of induction are likely to benefit from serial MRD monitoring by RT-PCR for a period of 3 years from completion of therapy...

..HSCT would be the treatment of choice in children with severe aplastic anemia who have a human leukocyte antigen (HLA)-matched related donor and is superior to IST in this series from India...

..8, P = .008). These results suggest that the PC dendritic cell count in the peripheral blood on day 28 is a strong predictor for development of GVHD in recipients of an allogeneic matched related PBSCT...

..Apart from describing a significant number of novel mutations, this report is the first study from Southern India to describe FXIII A gene mutations...

..Even in resource-constrained environments, education and a policy of systematic screening of patients associated with judicious use of bypassing agents can significantly improve the care of PWH who develop inhibitors...

..One patient had a granulocytic sarcoma. Complete remission was achieved in 96% of the 26 newly diagnosed patients after first induction. The median follow-up was 25 months (range 4-68). The overall survival was 69% at 31 months...

..Limited data exists on the long-term treatment outcome and prognosis of childhood ALL in India...

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..Patients in this high-risk group would probably benefit from novel therapeutic approaches...

..Prevention programs based on molecular diagnosis of heterozygous carriers and/or patients require the use of reliable mutation scanning methods in at-risk populations...

..We hope that this experience will form a framework on which guidelines can be established for developing countries...

..The profile and mortality of infections in this series from India is not significantly different from reports from the West...

..We also describe a possible misdiagnosis in the heterozygous state of Hb Lepore, as Hb Lepore and Hb A2 are not distinctly separated by cation exchange high performance liquid chromatography...

..While each signal pattern is typically associated with a particular abnormality, there can be more than one explanation for each pattern. Hence, metaphase FISH analysis is the "gold standard" for the interpretation of signal patterns...

..In our series, HSTCL was characterized by typical clinical and variable immunophenotypic features and a dismal clinical outcome...

..Other novel variants in TPMT or variations in the genes involved in transport and biotransformation of 6-MP need to be evaluated in the Indian population...

..Direct and inverted repeat sequences present in the deleted region might be involved in the origin of this mutation. The patient had moderate anemia and did not require blood transfusions (thalassemia intermedia)...

..The response to chemotherapy was poor in the remaining patients. The only patients who survived were three out of the four who had allogeneic hematopoietic stem cell transplantation (HSCT)...

..This study shows that cases of Hb E/Lepore may remain undetected unless family studies and/or detailed DNA analyses in patients diagnosed to have Hb E-beta-thal are performed...

..A study of the UGT1A1 gene polymorphism shows that the variant TA7 in the promoter region of the UGT1A1 gene is associated with hyperbilirubinemia in homozygous HbE patients...

..The mutation producing this abnormal hemoglobin (Hb) was found on the same haplotype in all these patients but differed from the Japanese haplotype, indicating its independent origin in India...

..8; 95% CI, 1.06-2.93; P = .028). These data suggest that outcome after allogeneic stem cell transplantation could be affected by many factors. The mechanisms by which they bring about such impact needs further evaluation...

..In this report, we explain the process and steps involved in obtaining a valid lab-specific conversion factor for expressing BCR-ABL1 transcript levels in the IS...

..A detailed understanding of all factors that may contribute to joint damage in severe hemophilia could help us in tailoring therapy for these individuals...

..012) and a delay in achieving a molecular remission (p=0.022). Neither FLT3 activating mutations nor secondary cytogenetic changes had an impact on clinical outcome...

..We evaluated factors influencing variation in CDA mRNA expression in adult AML patients and normal controls, and how they contributed to Ara-C cytotoxicity in AML cells...

..36.3%; p = 0.133) and improved event free (85.7 vs. 54.5%; p = 0.177) and overall survival (85.7 vs. 63.6%; p = 0.350). Flu with Cy +/- ATG reduces rejection and improves overall and event free survival in children with aplastic anemia...

..We have also shown that modest doses of intermediate purity FVIII (Koate DVI; Talecris Biotherapeutics, Raleigh, NC, USA) at 35 IU/kg preoperatively and 10 to 20 IU/kg after that are sufficient for surgical hemostasis in these patients...

..In this review we focus on the use of single agent ATO for the treatment of APL and summarize our experience and review the literature...

..These mutations were considered to be causative of disease because of their nature, evolutionary conservation and molecular modeling. This is the first report of mutations in patients with FVII deficiency from southern India...

..A slipped strand mispairing during DNA replication repair is proposed as the potential mechanism in generating this small deletion...

..No studies so far have examined enteric infections in patients undergoing bone marrow transplantation (BMT) in developing countries where asymptomatic carriage and colonization with enteric pathogens is frequent...

..In our experience, arsenic trioxide is effective in inducing and maintaining remission in patients with APML with minimal side effects. The optimal regimen and total dose required need to be defined...

..8 fold increase in transgene expression. Collectively, these data suggest that AAV vectors activate the cellular UPR pathways and their selective inhibition may be beneficial during AAV mediated gene transfer...

..This article reviews the applications of molecular genetics in hemophilia, in general, and how such techniques can be useful for optimizing patient care, in particular...

..The challenges ahead are to increase participation, improve reporting of results, and provide individualized support to laboratories to improve performance when necessary...

..A review of pediatric NHL statistics from population-based cancer registries was included in this study...

..Such improved understanding should assist with the development of contextually relevant guidelines for the management of hemophilia...

..The effect of intralesional mesenchymal stromal cell (MSC) transplant in the subacute phase of brain injury has not been studied...

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..Though only 5.5% achieved molecular remission at induction remission, 94.5% became negative during consolidation. At 20 months follow-up, 85% remain in remission but longer follow up studies are needed to monitor late relapses...

..This study extend our previous finding that mutations responsible for type 3 VWD are scattered throughout the entire VWF gene and that there is no founder effect in these three populations studied...

..We demonstrate that endothelial cells, contrary to hepatic cells do not express GST alpha either at mRNA or protein levels and hence are potentially susceptible to busulfan-mediated cytotoxic damage...

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..The mean value for indoor radon level given in the report of UNSCEAR 2000 for India needs to be revised...