Iranian Journal of Blood and Cancer
Volume:9 Issue: 4, Dec 2017

Blood pressure in patients with sickle cell anemia (SCA) is influenced by autonomic cardiovascular dysfunction, endocrinopathies, nephropathy and nutritional factors. We aimed to evaluate systemic and diastolic blood pressure and its severity among children and adolescents with SCA and determine its association with clinical and hematological variables.
This case-control study included 112 patients with SCA (6-17 years old), registered at Basra Center for Hereditary Blood Diseases and 133 age and sex-matched healthy subjects as a control group. Complete examination was done for all subjects including anthropometric and BP measurements. Complete blood count, blood urea and serum creatinine were measured for all participants. A multivariate correlation analysis was used to assess contributing factors in BP.
The systolic BP (SBP) and diastolic BP (DBP) curves of patients with SCA showed lower levels than control group for all age groups. Although both SBP and DBP readings were comparable at late adolescence. Pre-hypertension was reported in 5 (4.4%) patients and 3 (2.4%) healthy subjects. Multivariate analysis revealed that SBP and DBP among patients with SCA had significant positive association with age, weight, height, body mass index, hemoglobin and blood transfusion history and a negative association with heart rate ( P Our findings supported previous reports of lower BP in patients with SCA. Furthermore, routine BP measurements in pediatric patients with SCA is important for early detection of pre-hypertension to establish an effective treatment.

Deferoxamine mesylate is still the conventional and well-known iron chelator for patients with thalassemia major. However, due to some marketing issues the well-known original brand, Desferal®, produced by Novartis Pharmaceuticals Company is not as available as before. The generic brands of Deferoxamine have been introduced in many parts of the world including Iran; however, they are not well accepted by the physicians and patients yet. This triple-blind randomized controlled trial was designed to compare the efficacy and safety of a new Iranian generic and the original brand product of Deferoxamine mesylate in Iranian patients.
The present Randomized triple-blind controlled trial research was carried on in nine centers throughout Iran. They were randomly divided into two similar groups and a Cross-over study was designed. 24-hour urine was collected after subcutaneous infusion of either drugs and urinary iron excretion was measured via atomic absorption spectrophotometer device. Acute adverse events during and after drug infusion were recorded. Mack Nara test and p-pair test were applied to compare two cross over interventions.
154 patients from 9 centers were enrolled in this study. There were 95 women and 59 men aged 6-34 years (mean age of 21.1years). Mean urinary iron concentration for Desferal (intervention A) vs. Desfonak (intervention B) groups was 22.5±22.6 vs. 21.5±16.9 mg/m2 , respectively. Mean urinary iron excretion/Kg body weight for Desferal (intervention A) vs. Desfonak (intervention B) groups was 0.48±0.48 vs. 0.47±0.40 mg/m2, respectively.
According to the results of this study, there was similarity between efficacy and safety of the original and generic brands of deferoxamine (desferal vs. desfonac).

Iron deficiency, is a common worldwide complication, especially in preschool children. Iron deficiency anemia (IDA) is associated with changes in the mental and motor development, defects of immune system, cardiovascular, diseases and disruption of lipid profile. In IDA, the occurrence of dyslipidemia is controversial. We aimed to investigate the association between IDA and serum lipid concentration.
In this comparative cross-sectional study, 41 children with confimed IDA and 39 healthy children were evaluated. Hemoglobin, Hematocrit, serum ferritin level, triglyceride (TG), total cholesterol (TC), low density lipoprotein (LDL), high density lipoprotein (HDL) and anthropometric indices including weight, height, and body mass index were assessed.
The mean±SD age of children with IDA and control group were 10.7±3.5 years and 9.5±3.2 years, respectively (P=0.45). Mean±SD serum lipid profile in control vs IDA groups were as follows: TG (86.1±36.9 vs 88.6±33.5 mg/dl), TC (151.7±28.7 vs 148.9±29.2 mg/dl), LDL (78.4±29.1 vs 88.1±22.6 mg/dl) and HDL (54.2±13.1 vs 52.5±10.1 mg/dl). The results showed no statistically significant difference between IDA and control groups. Slightly increased but not significant TG and LDL levels were observed in the IDA group compared with the control group.
Theses findings indicated that there was no significant difference between IDA and control group in terms of serum lipid concentrations.

Acute lymphoblastic leukemia (ALL) is the most common childhood cancer. Several studies have shown that ALL occurs as a result of genetic abnormalities. 1, 25-Dihydroxyvitamin D3 as a secosteroid hormone plays an important role in different metabolic pathways. The normal function of vitamin D occurs via binding to a ligand-activated transcription factor i.e. vitamin D receptor (VDR). Therefore genetic variation in VDR may lead to various disorders, including cancer. We aimed to investigate rs731236 polymorphism of VDR gene in children with ALL.
Genomic DNA was extracted from 50 children under 15 years of age with ALL and 50 age-matched healthy children’s whole blood and genetic variation of each participant was detected using polymerase chain reaction and restriction fragment length polymorphism (PCR-RFLP) with taqI restriction enzyme. Statistical analysis was performed using SPSS v.22 following χ2 test.
The VDR gene polymorphisms were genotyped in a total of 50 individuals with ALL, comparing 50 normal children with a mean age of 5.2±3.4 years. No deviation was observed from Hardy-Weinberg equilibrium (HWE) in the genotypic distribution of the rs731236 (χ2=0.25, P>0.05). Genotype frequency of TT (Dominant Homozygote), Tt (Heterozygote), and tt (Recessive Homozygote), was 25, 15 and 10 in the case group and 21, 27 and 2 in the control group, respectively which showed a significant difference between two groups (P=0.011). tt genotype showed a strong protective effect against ALL over Tt (OR=9, CI 95%: 1.74-46.59).
We have determined the frequency of Taq1 (rs731236) polymorphism in the VDR gene in children with ALL in Zanjan population. We concluded that genotype variation in VDR gene may have an effect on incidence of ALL.

Oxidants and inflammation agents are predisposing factors for the development of prostate cancer. As a chemical warfare, sulfur mustard (SM) can cause cancer through various pathways mainly increased production of oxidants and inflammation. Due to high incidence of cancer in SM victims, concentration of prostate specific antigen (PSA) in SM victims was evaluated and compared with the control group.
This study was conducted on 150 subjects exposed to SM as the Iranian chemical victim group and 150 non-exposed healthy subjects matched for age and sex, as the control group. The serum concentration of PSA was measured by Enzyme-linked Immunosorbent assay.
According to spirometry results, the SM exposure rate in the chemical victim group was mild, moderate, and severe in 66%, 27% and 7%, respectively. The mean time elapsed from exposure to SM in case group was 30 years. 100% of the victims had consumed N-acetylcysteine for alleviating symptoms due to exposure to SM. Decreased concentration of PSA in SM victims compared with control group was observed; even non-significant. (0.728 vs 0.844 ng/ml, P=0.103).
PSA concentrations were expected to increase in SM victims, but in our study an opposite result was observed. It is assumed that consumption of N-acetylcysteine with known anti-inflammatory features, mild exposure and shorter period of time elapsed from exposure to SM may be the reasons for this results. Further studies on these subjects seem to be necessary to prove the efficacy of antioxidants such as N-acetylcysteine in prevention of prostate cancer in subjects who have the history of SM exposure.

Gastric carcinosarcoma is a rare cancer of adults with poor prognosis compared with other gastric malignancies. To the best of our knowledge, the present report is the first case of gastric carcinosarcoma with chondroblastic differentiation in Iran. A 63-year-old man with epigastric pain was admitted to the surgery department. Abdominopelvic CT-scan showed a hypodense mass lesion in distal gastric lesser curvature measuring 5.7 x 4.0 cm with decreased enhancement after contrast injection. Microscopic examination revealed coexistence of carcinomatous and sarcomatous components, presence of vascular and perineural invasion and involvement of the adjacent lymph nodes. This is a report of a rare case of gastric carcinosarcoma with chondroblastic differentiation.

We present a 34-year-old man with combination of beta thalassemia intermedia and coagulation factor VII deficiency who was presented with pallor and irregular nose bleeding episodes. On physical examination, he had splenomegaly and yellow sclera. Pallor and splenomegaly could be reminder of thalassemic syndromes or hemoglobinopathies including thalassemia intermedia. Association with unusual bleeding tendency such as prolonged and repeated episodes of epistaxis without any evidence of liver failure in hemoglobinopathies suggest the possibility of coinheritance of a bleeding disorder. As a result, coagulation assay for proper diagnosis, management and prevention of probable life-threatening bleeding episodes is suggested.