Iranian Journal of Blood and Cancer
Volume:10 Issue: 1, Mar 2018

Although several studies have been done on quality of life of patients with Sickle Cell Disease (SCD), there is little research on the correlation of health-related quality of life (HRQoL) with self-efficacy in these patients. We aimed to determine the association between HRQoL and self-efficacy in patients with SCD and to explore the role of self-efficacy and demographic-clinical variables in a sample of Iranian patients with SCD.
In this cross-sectional study, 97 SCD patients who had medical records in Shafa Hospital affiliated to the Ahvaz University of Medical Sciences were recruited. Data were collected using the Short-Form Health Survey SF-36 (RAND 36-item), the Sickle Cell Self-Efficacy Scale (SCSES) and a demographic-clinical information questionnaire during February to July 2013.
The mean scores of physical and mental component summary of the SF-36 (PCS and MCS) was 45.58±19.94 and 48.1±19.63, respectively which were low in patients with SCD. Moreover,
50.5% of the patients reported a moderate level of self-efficacy (24.42±6.59). Regression models showed that self-efficacy was the most important predictor of the mental component summary (MCS) (β: 0.48, P=0.001). With a slight difference, it was the second strongest predictor of the physical component summary (PCS) (β: 0.28, P=0.003), after the variable of “renal disease history” in the context of SCD (β: -0.30, P=0.001). However, “blood transfusion history” was a common predictor for both the PCS (β: 0.20, P=0.03) and the MCS (β: 0.26, P=0.001) components of the HRQoL.
The results of this study can assist health policy makers and clinicians to plan holistic interventions by focusing on the level of self-efficacy in SCD patients.

In patients undergoing chemotherapy for cancer, anemia is part of the progression of the disease. Considering the effects of anemia on quality of life of the patients, the prevention and treatment of chemotherapy-induced anemia is crucial. This study was aimed to evaluate the efficacy of recombinant human erythropoietin in reducing the need for blood transfusion in children with solid tumors receiving chemotherapy.
In a clinical trial, 57 children referred to the pediatric centre of Tabriz University of Medical Sciences with a diagnosis of solid tumor were randomly assigned into two groups. The intervention group (n=29) received recombinant human erythropoietin (rHuEPO) at a dose of 450 IU/kg subcutaneously once a week for 12 weeks, and the control group (n=28) received no intervention in this regard. Hemoglobin levels were analysed at the beginning and end of the study. The need for blood transfusion was also assessed in the patients.
The mean Hb at the beginning of the study was 8.85±1.01 and 8.98±0.11 g in the intervention and control groups, respectively. The mean Hb at the end of the study was 9.78±0.32 g/dl in the intervention group and 7.79±0.24 g/dl in the control group. Hb level was significantly higher in the intervention group at the end of the 12 weeks of treatment with rHuEPO than in the control group (P=0.001).
Based on the results of this study, rHuEPO administration seems to be beneficial in the prevention and treatment of chemotherapy-induced anemia in children with solid tumors and reduces the amount of blood transfusion in these patients.

Late onset neutropenia (neutropenia after 3 weeks of life) may be a physiological condition without need to prescribe antibiotics, G-CSF, or IVIG. We aimed to determine the association between sepsis and late onset neutropenia in very low birth weight ( This study was a cross-sectional prospective study in VLBW infants that were admitted in Mahdieh Hospital in Tehran/Iran. Complete blood count (CBC) was drawn at first day of admission and then weekly intervals until discharge from the hospital. In cases with neutropenia, CRP and blood cultures were assessed and correlation between clinical sepsis or positive blood culture and CRP with neutropenia was evaluated.
219 VLBW infants during a period of 11 weeks were studied with serial weekly CBCs. 128 (58%) neonates had normal neutrophil counts, 91 (41.6%) had neutropenia, of which 28 (30.7%) had late onset neutropenia. Mean level of WBC was 5033.07±2037.57 cells/mm3 and mean level of ANC was 1757.75±944.32 cells/mm3. Positive blood culture at first week of life had significant correlation with neutropenia (P=0.001), but there was no significant correlation between late onset neutropenia and positive CRP (P=0.861), clinical symptoms of sepsis (P=0.5) and bacteremia (P=0.861). There was significant correlation between anemia (P VLBW infants admitted to the NICU with late onset neutropenia without symptoms of sepsis do not need any intervention and close observation with follow-up would be the most appropriate approach.

Portulaca oleracea as a herbal plant has been widely used in traditional medicine to cure many diseases. The therapeutic effects of this plant are exerted through its active ingredients with antioxidant properties. It has been shown that this plant may have cytotoxic effects on cancer cells. In the present study, cytotoxic effects of aqueous extract of Portulaca oleracea on oral cavity cancer cell KB cell line has been investigated.
KB cancer cell line was purchased from National Cell Bank of Pasteur Institute of Iran. Aqueous extract of P. oleracea was prepared by the maceration method. Cytotoxic effect of the aqueous extract of P. oleracea was assessed through different doses for 12, 24 and 48 hours using 3-(4,5-Dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT ) assay and Trypan blue. Also, morphological changes of the nucleus were carried out by Hoechst Staining.
Rate of proliferation in cells treated with P.olarecea aqueous showed a significant decrease depending on dose and time; as with the increase of the dose and time, the more cells were decreased. There were also nuclear changes indicative of apoptosis in the cells treated with the extract.
It seems that P.oleracea aqueous extract has anti-proliferative effects on oral cavity KB cancer cell line. Further studies need to be undertaken to prove the efficacy and mechanism of its action.

Pancytopenia in childhood can be caused by a variety of underlying diseases including hematological and non-hematological entities. Phenylketonuria (PKU) is an inborn error of phenylalanine metabolism. No association between PKU and pancytopenia has ever been reported. We report the first case of PKU revealed by a pancytopenia at presentation. The patient was an infant girl born to healthy non-consanguineous parents with unremarkable family history. A hereditary metabolic disease workup was performed due to the presence of unexplained hematological features and a global developmental delay. Plasma aminoacid profile by thin-layer chromatography showed elevation of phenylalanine and urine organic acid chromatography showed accumulation of metabolites of phenylalanine; whereas, methylmalonic acid or other abnormal organic acids were not found. This is the first case of untreated PKU associated with pancytopenia who improved with low-phenylalanine diet.

Extramedullary hematopoiesis (EMH) is a physiological compensatory phenomenon occurring in many hemolytic anemias including thalassemia. Besides transfusion, radiotherapy, surgery or a combination of these modalities, hydroxyurea (HU) as an optimal treatment has been described occasionally. We described a case of beta-thalassemia major who has been on regular blood transfusion and developed EMH that was treated with HU combined with radiotherapy.
EMH should also be considered in thalassemia major patients, although it is more common in non-transfusion dependent thalassemia patients. HU combined with low dose radiotherapy along with regular blood transfusion are suggested as good treatment options for patients with EMH.