The Impact of Prophylactic Post-Chemotherapy G-CSF on the Relapse Rates in Patients with Acute Myeloid Leukemia: A Meta-Analysis.

This meta-analysis evaluated the impact of prophylactic post-chemotherapy granulocyte colony-stimulating factor (G-CSF) in patients with acute myeloid leukemia (AML). Overall, the relapse rate, overall survival, event-free survival, and mortality rate were similar in G-CSF (+) compared to G-CSF (-) patients. However, the relative risk (RR) of relapse was higher in children and in secondary AML patients who were treated with G-CSF compared to the G-CSF (-) group [RR, 95% confidence interval: 1.26, 1.04-1.52, and 1.12 (1.02-1.24)]. Treatment with post-chemotherapy G-CSF should be prescribed with caution in pediatric patients with AML and secondary AML as possibly increasing the relapse risk.

Evaluation of the efficacy of signal-averaged electrocardiogram testing in the cardiac assessment of beta-thalassemia major patients.

BACKGROUND: More than 70% of thalassemia's major mortality is due to the cardiac complications of this syndrome, mostly consequent to myocardial Iron overload; therefore, evaluation of such complications is of utmost importance. T2*MRI is used to assess hepatic and myocardial Iron load in thalassemia patients, which is not always available. Signal-Averaged Electrocardiography is a rather easy method of evaluating major thalassemia patients regarding their risk for sudden cardiac death. METHODS AND MATERIALS: In this cross-sectional study, 48 patients with thalassemia major underwent evaluation with electrocardiography, signal-averaged electrocardiography, echocardiography, T2*MRI, and ferritin level. The association of the existence of ventricular late potentials in SAECG and other cardiac variables was evaluated. Moreover, the association between myocardial and hepatic Iron load and cardiac characteristics was assessed. RESULTS: 48 patients with a mean age of 30.31 ± 7.22 years old entered the study. 27 (56.3%) of the patients had ventricular late potentials, which were associated with myocardial dry Iron weight (P = 0.011). Nonspecific ST-T changes and premature atrial and ventricular contractions were seen more frequently in patients with late potentials (P = 0.002, 0.031, and 0.031, respectively). Patients with higher myocardial and hepatic Iron loads had longer QTc in their 12-lead surface electrocardiograms. CONCLUSION: Patients with ventricular late potentials assessed by SAECG had a higher myocardial Iron load. Higher myocardial Iron load is associated with higher cardiac complications in patients with beta-thalassemia major; therefore, SAECG can be used as a screening test for cardiac complications in beta-thalassemia major patients.

The Outcome of Children With Malignant Bone Tumors: A Single-Center Experience.

Malignant bone tumors (MBT) account for 3% to 5% of cancers in children younger than 15 years. We aimed to report the outcome of children with MBT in 10 years in Southern Iran. During the study period, 100 patients (57 Osteosarcoma, 43 Ewing sarcoma) with an M/F ratio of 1.56 and a median age of 13.8 years (3.8-17.9) were diagnosed. Metastasis occurred in 27% of patients, mostly in the first 3 months of diagnosis. The mean survival time of MBT altogether was 94.1 months (95% CI: 86.5-101.7). The 5-year overall survival and event-free survivals were 85.2% (95% CI: 74%-91.8%) and 69.2% (95% CI: 56%-79%), respectively. Metastasis was the only independent risk factor of death in our study cohort (Hazard ratio 36.7, 95% CI: 4.8-282.6, P = .001) MBT in children mostly occur in adolescent boys. About one-third of them become metastatic, which is significantly associated with poor outcomes.

Potential voriconazole associated posterior reversible leukoencephalopathy in children with malignancies: Report of two cases.

INTRODUCTION: The fungal infection has become severe morbidity amongst patients with malignancy. Voriconazole, a new generation of triazole, has shown excellent results in treating invasive fungal infections. CASE REPORT: Herein, we report two cases of posterior reversible encephalopathy syndrome (PRES), which induced after voriconazole exposure.Management and outcome: Magnetic resonance imaging, and the serum level of voriconazole were investigated in both patients to assess toxicity. The role of methotrexate, as one of the possible causes of PRES, is weakened significantly through precise assessing diffusion-weighted images on magnetic resonance imaging. DISCUSSION: These unique cases emphasize that voriconazole can induce PRES even at therapeutic levels. Therefore, in the case of neurotoxicity, PRES must be considered, and voriconazole should discontinue. The prognosis seemed promising when voriconazole stopped immediately after clinical suspicion.

Bone mineral density in transfusion-dependent thalassemia patients and its associated factors in Southern Iran.

Despite the significant improvements in the management of thalassemia, there are growing concerns regarding their long-term complications. We showed that low bone mass is one of the most prevalent complications among these patients. Insufficient physical activity and hypogonadism are the main possible associated factors followed by DM and insufficient sun exposure. PURPOSE: Despite the significant improvements in the management of transfusion-dependent thalassemia (TDT), there are growing concerns regarding their long-term complications. METHODS: This cross-sectional study included 615 TDT patients who were registered and followed in a comprehensive thalassemia clinic in Southern Iran. We measured serum biochemical tests and bone mineral density in all patients. We recorded physical activity and sun exposure subjectively, and an endocrinologist visited and examined all the patients. A group of age- and gender-matched healthy volunteers participated in the study as the control group. RESULTS: The mean age of the studied population was 28.4 ± 7.7 years, and 55.8% were female. The prevalence of vitamin D deficiency was 45.6% and 54.4% in TDT patients and the control group. A portion of TDT patients suffered from different endocrinopathies, which included hypogonadism (49.8%), diabetes mellitus (17.2%), hypoparathyroidism (14.6%), and hypothyroidism (6.3%). The prevalence of low bone mass in patients with TDT was 48.3 and 74.6% in the femoral and lumbar bones, respectively. Low physical activity, insufficient sun exposure, diabetes mellitus, and hypogonadism were associated with low bone mass. CONCLUSION: Low bone mass is highly prevalent among TDT patients in Southern Iran. Insufficient physical activity and hypogonadism are the main possible associated factors, followed by DM and insufficient sun exposure.

Bleeding symptoms in patients diagnosed as type 3 von Willebrand disease: Results from 3WINTERS-IPS, an international and collaborative cross-sectional study.

BACKGROUND: Type 3 von Willebrand's disease (VWD) patients present markedly reduced levels of von Willebrand factor and factor VIII. Because of its rarity, the bleeding phenotype of type 3 VWD is poorly described, as compared to type 1 VWD. AIMS: To evaluate the frequency and the severity of bleeding symptoms across age and sex groups in type 3 patients and to compare these with those observed in type 1 VWD patients to investigate any possible clustering of bleeding symptoms within type 3 patients. METHODS: We compared the bleeding phenotype and computed the bleeding score (BS) using the MCMDM-1VWD bleeding questionnaire in patients enrolled in the 3WINTERS-IPS and MCMDM-1VWD studies. RESULTS: In 223 unrelated type 3 VWD patients, both the BS and the number of clinically relevant bleeding symptoms were increased in type 3 as compared to type 1 VWD patients (15 versus 6 and 5 versus 3). Intracranial bleeding, oral cavity, hemarthroses, and deep hematomas were at least five-fold over-represented in type 3 VWD. A more severe bleeding phenotype was evident in patients having von Willebrand factor antigen levels < 20 IU/dL at diagnosis in the two merged cohorts. In type 3 patients, there was an apparent clustering of hemarthrosis with gastrointestinal bleeding and epistaxis, whereas bleeding after surgery or tooth extraction clusters with oral bleeding and menorrhagia. CONCLUSIONS: In the largest cohort of type 3 VWD patients, we were able to describe a distinct clinical phenotype that is associated with the presence of a more severe hemostatic defect.

Efficacy and safety of resveratrol, an oral hemoglobin F-augmenting agent, in patients with beta-thalassemia intermedia.

Recently, resveratrol showed induction of γ-globin mRNA synthesis in human erythroid precursors and reducing oxidative stress in red cells of thalassemia patients in many in vitro studies. We aimed to investigate the efficacy and safety of resveratrol, for the first time, in non-transfusion-dependent beta-thalassemia intermedia (B-TI) in Southern Iran. In this double-blind randomized clinical trial, 54 patients with B-TI were investigated during 6 months between October 2016 and March 2017. Patients were randomly allocated into three groups by simple randomization method. Group 1 (hydroxyurea (HU) and placebo, 18 patients), group 2 (resveratrol/piperine and placebo, 16 patients), and group 3(HU and resveratrol/piperine, 20 patients). Primary end point was considered as change in hemoglobin (Hb) levels and need for blood transfusion. Drug safety was considered as a secondary end point. Mean age of the patients was 28.2 ± 5.6 (18-42) years. Response rate was not significantly different among the three groups (P > 0.05). Higher percentages of adverse events were detected in groups 2 (31.3%) and 3 (25%) compared to group 1 (5.6%). However, the difference was not statistically significant (P > 0.05). All reported adverse events were gastrointestinal symptoms. Resveratrol showed a similar efficacy with HU in the small population of non-transfusion B-TI patients during a 6-month follow-up. Complications, mostly gastrointestinal, were observed more frequently in resveratrol groups compared to the HU group. Although it was not statistically significant, more attention should be given to safety and efficacy of resveratrol as an oral HbF-augmenting agent.

Complementary and alternative medicine use in thalassemia patients in Shiraz, southern Iran: A cross-sectional study.

This study aimed to determine the frequency and pattern of complementary and alternative medicine (CAM) use in thalassemia patients in south of Iran. The survey was done using a validated questionnaire which was distributed among 122 thalassemia patients. Only 108 questionnaires were completed and turned back (response rate 88.5%). Patients referred to an outpatient thalassemia clinic in Shiraz, southern Iran for blood transfusion. The mean age of the patients was 22.9 ± 7.9 years (range 4-45 years) with female/male ratio 1.84. Seventy four (68.5%) of the responders used CAM at least once during their life, and about half of them used it concurrently with their conventional treatments. The most reported CAM product was mint juice (50%). The most common reason of CAM use was increased general health. The most common information source about CAM was physicians who were the most trusted source as well. CAM is frequently being used in thalassemia patients to ensure their sense of well-being and help them overcome the complications of their illnesses.

Wound Healing Studies Using Punica granatum Peel: An Animal Experimental Study.

OBJECTIVE: The goal of the present study was to evaluate the effects of hydroalcoholic extract-based carboxy methyl cellulose (CMC) gel of Punica granatum peel (PCMC) and CMC on healing of full-thickness skin wounds. MATERIALS AND METHODS: Forty-two rats were studied. Each rat had 3 wounds that were treated topically with PCMC as the case, CMC as the positive control, and sterile saline as the negative control. All 3 wounds of each rat were photographed during the wound healing period at days 0 (onset of wound surgery), 3, 6, 9, and 12.The wound area was calculated using Adobe Photoshop CS (version 5) software (Adobe Systems Inc, San Jose, California). Electrocardiogram paper was used for reference scale. RESULTS: The results of this study show that macroscopic and microscopic wound healing took a significantly longer time in wounds treated with normal saline than those treated with PCMC (grossly) and CMC gel (grossly and significantly). CONCLUSIONS: The authors' findings show that anti-inflammatory, antihemorrhagic, and antinecrotic effects of CMC lead to early healing of skin wounds.