Presacral and intrathoracic extramedullary hematopoiesis: a case report.

Extramedullary hematopoiesis (EMH) refers to the location of hematopoietic elements in locations other than the bone marrow and peripheral blood. It may be seen as a compensatory condition in many hematological conditions including thalassemia, or it may occur as an incidental finding. Intrathoracic EMH commonly develops in the posteroinferior mediastinum. Presacral EMH is an extremely rare condition, and there are a limited number of case reports published in the literature. We are reporting a case of EMH that involves the posterior mediastinum and presacral region on computed tomography and magnetic resonance imaging in a patient with thalassemia intermedia

Prevalence and Risk Factors of Anemia among Adolescents in Denizli, Turkey.

OBJECTIVE: The aim of this study was to evaluate the prevalence and risk factors of anemia among adolescents in Denizli where Mediterranean cuisine (fresh fruit and vegetables) is adopted. METHODS: We accepted hemoglobin values below 12 g/dl for girls and 13 g/dl for boys as the criteria of anemia. We recorded a detailed history including nutritional habits, consumption of animal source foods especially red meat, consumption of fresh fruit and vegetables, presence of parasitic infestation, psychosocial status, school success and any materials consumed except ordinary food. FINDINGS: We investigated the prevalence of anemia in 1120 children (672 girls and 448 boys), aged 12 to 16 years. We found that the overall prevalence of anemia was 5.6%. 8.3% of the girls and 1.6% of the boys were anemic. We diagnosed iron deficiency anemia in 37(59%) anemic patients and combined iron deficiency and vitamin B12 deficiency anemia in 26 (41%) anemic patients. None of the patients had folic acid deficiency. CONCLUSION: Our results suggest that the socioeconomic status of the family, traditional eating habits of the region, the fear of gaining weight and irregular eating habits are of great importance in the development of adolescent anemia in Denizli

Relation between 3435C>T multidrug resistance 1 gene polymorphism with high dose methylprednisolone treatment of childhood acute idiopathic thrombocytopenic purpura

The current study was conducted to assess 3435C>T multidrug resistance 1 gene polymorphism and the efficacy of high dose methylprednisolone (HDMP) in childhood acute idiopathic thrombocytopenic purpura patients. Methods: A total of 31 childhood acute Idiopathic thrombocytopenic purpura patients (17 females, 14 males) between the ages of 2 and 16 years of age were included in the study. High-dose methylprednisolone was given at a dose of 30 mg/kg/day for 3 days and 20 mg/kg/day for 4 days, consecutively and intravenously. Polymerase chain reaction-restriction fragment length polymorphism was used for the detection of C3435T single nucleotide polymorphism. Fragments obtained were 238 bp to T/T genotype, 172 bp and 60 bp fragments to the C/C genotype, and 238 bp, 172 bp and 60 bp to the C/T genotype. Results: The distribution of CC, CT, and TT genotypes were 19.0%, 61.3%, and 19.4%, respectively. Both allele frequencies of C and T were the same - 50%. There was no significant difference in genotype and allele distribution between the patients with ITP and the control group (Χ 2=0.84 p=0.65, Χ 2=0.2 p=0.63, respectively). There were no significant differences in age, gender, and pre- and post-treatment platelet counts between CC, CT, and TT genotypes of the MDR gene. Response to treatment shows no significant difference between genotype and allele groups. Conclusion: In our study, there was no difference in the HDMP treatment response between MDR1 gene genotypes. However, it should be noted that this study includes a small group of patients. Our data should therefore be considered preliminary, awaiting further confirmatory studies on an expanded patient base. © 2011 Elsevier B.V

Deferasirox in children with transfusion-dependent thalassemia or sickle cell anemia: A large cohort real-life experience from Turkey (REACH-THEM)

Objectives: To evaluate the long-term efficacy and safety of deferasirox therapy in a large observational cohort of children with transfusion-dependent thalassemia (TDT) and sickle cell anemia (SCA) in Turkey. Methods: This was a multicenter, prospective cohort study including TDT and SCA patients aged 2-18 years with iron overload (≥100 mL/kg of pRBC or a serum ferritin [SF] level >1000 μg/L) receiving deferasirox. Patients were followed for up to 3 years according to standard practice. Results: A total of 439 patients were evaluated (415 [94.5%] TDT, 143 [32.6%] between 2 and 6 years). Serum ferritin levels consistently and significantly decreased across 3 years of deferasirox therapy from a median of 1775.5 to 1250.5 μg/L (P < 0.001). Serum ferritin decreases were noted in TDT (1804.9 to 1241 μg/L), SCA (1655.5 to 1260 μg/L), and across age groups of 2-6 years (1971.5 to 1499 μg/L), 7-12 years (1688.5 to 1159.8 μg/L), and 13-18 years (1496.5 to 1107 μg/L). Serum ferritin decreases were also noted for all deferasirox dose groups but only significant in patients with doses ≥30 mg/kg/d (n = 120, −579.6 median reduction, P < 0.001). Only 9 (2%) patients had adverse events suspected to be related to deferasirox. Serum creatinine slightly increased but remained within the normal range. Conclusions: Deferasirox has long-term efficacy and safety in children with TDT and SCA, although higher doses (≥30 mg/kg/d) may be required to achieve iron balance. © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Lt

Deferasirox in children with transfusion-dependent thalassemia or sickle cell anemia: A large cohort real-life experience from Turkey (REACH-THEM).

OBJECTIVES: To evaluate the long-term efficacy and safety of deferasirox therapy in a large observational cohort of children with transfusion-dependent thalassemia (TDT) and sickle cell anemia (SCA) in Turkey. METHODS: This was a multicenter, prospective cohort study including TDT and SCA patients aged 2-18 years with iron overload (≥100 mL/kg of pRBC or a serum ferritin [SF] level >1000 μg/L) receiving deferasirox. Patients were followed for up to 3 years according to standard practice. RESULTS: A total of 439 patients were evaluated (415 [94.5%] TDT, 143 [32.6%] between 2 and 6 years). Serum ferritin levels consistently and significantly decreased across 3 years of deferasirox therapy from a median of 1775.5 to 1250.5 μg/L (P < 0.001). Serum ferritin decreases were noted in TDT (1804.9 to 1241 μg/L), SCA (1655.5 to 1260 μg/L), and across age groups of 2-6 years (1971.5 to 1499 μg/L), 7-12 years (1688.5 to 1159.8 μg/L), and 13-18 years (1496.5 to 1107 μg/L). Serum ferritin decreases were also noted for all deferasirox dose groups but only significant in patients with doses ≥30 mg/kg/d (n = 120, -579.6 median reduction, P < 0.001). Only 9 (2%) patients had adverse events suspected to be related to deferasirox. Serum creatinine slightly increased but remained within the normal range. CONCLUSIONS: Deferasirox has long-term efficacy and safety in children with TDT and SCA, although higher doses (≥30 mg/kg/d) may be required to achieve iron balance