Iranian Journal of Pediatric Hematology and Oncology
Volume:9 Issue: 3, Summer 2019

Many Red Blood Cell (RBC) indices have been developed based on mathematical formulae to discriminate beta-thalassemia trait (βTT) from iron deficiency anemia (IDA). The latter two conditions represent the most common causes of microcytic hypochromic anemia in Egypt. This study aimed To evaluate the diagnostic reliability of 24 published discriminant indices for differentiating βTT from IDA in Egyptians. A cross sectional study included a total of 166 subjects (108 IDA & 58 βTT) aged 1-18 years were recruited from Hematology laboratory of  Pediatric Hospital, Ain Shams University, Cairo, Egypt. A full diagnostic algorithm was performed using complete blood count, hemoglobin electrophoresis by High Performance Liquid Chromatography (HPLC), iron profile and PCR detection of 22 mutations common for βTT. Twenty-four formulas were applied and their performance characteristics were calculated for each index. The highest accuracy (True positive + True negative/ All cases) & Youden's Index (Sensitivity+Specificity-100) were for Red Cell Distribution Width (RDWI) and Hameed index closely followed by Keikhaei index while the least performance was for RDW-SD, RDW-CV and Shine & Lal indices. The superiority of an index over another in distinguishing βTT from IDA allowed only partially better selection of cases warranting further confirmatory molecular studies. None of the studied formulae provided a surrogate test for Hb electrophoresis as mass screening.

Platelets can activate B cells and stimulate them for the production of antibodies. Since platelet microparticles (PMPs) originate from platelets, they may have the same virtue. In the present study, the effect of PMPs was investigated on the production of human leukocyte antigen (HLA)-specific antibody from B cells in vitro. In this experimental study, HLA-DR antigen was solubilized from the immortalized B lymphocytes (Daudi cell line) and purified using the affinity chromatography. Antigen properties were determined by the ELISA technique. PMPs were isolated from platelet concentrate bags by centrifugation. Fresh blood products were prepared from the Innovation Center of Iranian Blood Transfusion Organization (IBTO) and B lymphocytes were purified by the MACS method. B cells were exposed with PMPs and HLA-DR antigens in the culture medium. On the third day of culture, the culture supernatant was examined in terms of antibody production using the ELISA test. The results were analyzed using paired sample T-test and P-value <0.05 was considered statistically significant. The specificity of the purified HLA-DR antigen was confirmed using the anti-HLA-DR antibody and ELISA technique in the presence of appropriate controls. The results showed that PMPs could stimulate the production of antibodies from B cells. The difference between the case and control was significant (P-value=0.001). Although total immunoglobulin (IgG) was higher in HLA-DR-treated wells, HLA-DR-specific antibodies were not identified by ELISA technique. PMPs have the capability to induce IgG antibodies from B cells. In order to ensure the production of specific antibodies, further testing is required with high sensitivity.

The changes of platelet parameters can be a useful index for rapid diagnosis of urinary tract infection (UTI), since platelet changes are routinely determined through complete blood count (CBC) test. The correlation between platelet indices, included number (PLTs), mean platelet volume (MPV) and platelet distribution width (PDW), which are the indicators of production and function of platelets, with UTI was evaluated in this study.         In this descriptive-analytical study, 97 patients with UTI (patient group) and 117 healthy people (control group). The average age for the patient and the control group was 10.84±6.68 and 11.34±7.1 years old, respectively. This study was done during 2016-2018 in Imam Reza Hospital, Kermanshah, west of Iran. The PLT, MPV, PDW, and other inflammatory indices, including white blood cell, neutrophils, lymphocytes, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) were evaluated. The diagnosis of bacteria was done using routine microbiological and biochemical methods. The platelet indices were statistically compared between the patients and the control groups (T test and Chi square test). The most common isolated gram negative and gram positive bacteria were E. coli, Citrobacter, and Staphylococcus aureus, respectively. In the patient group, PLT number was significantly higher than that in the control group (p=0.0007), while difference of other indices such as MPV, PDW, neutrophils, lymphocytes, CRP, and ESR were not statistically significant between the two groups. In case of UTI with gram positive bacteria, PLT number (p=0.05) was lower but MPV (p=0.02) and PDW (p=0.045) was higher compared to the UTI with gram negative bacteria. The results of this study showed that the platelet number could be a useful diagnostic index for urinary tract infection. However, more studies need to be done with higher number of patients to evaluate the more details of platelet changes during UTIs.

Storage of platelet concentrates (PCs) at room temperature (20-24°C) limits its storage time to 5 Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common inherited enzyme deficiency of the human red blood cells . Most of G6PD deficient individuals are asymptomatic, but acute hemolytic anemia may be presented with nausea, vomiting, abdominal pain, headache, jaundice, pallor, discoloration of the urine, chills, and fever. Seizure is reported as a rare symptom, as well. The present study aimed to investigate seizure following acute hemolysis caused by Glucose-6-phosphate dehydrogenase deficiency.   This analytic cross-sectional study was conducted on all consecutive patients aged 1-12 years with G6PD deficiency hospitalized for hemolysis in 17 Shahrivar children hospital, Rasht, Iran, in 2016. Demographic characteristics and other variables such as place of inhabitants, type of drinking water, history of seizure in the patients and family, cause of hemolysis, hemoglobin level and hemoglobinuria on admission, and infection history prior to hemolysis were recorded. Data were analyzed by Mann-Whitney U test and Fischer Exact Test.  P-value < 0.05 indicated statistical significance and data were assessed by SPSS (version 20).   The youngest patient was one year old and the oldest was 11 years old. Most of them were males (68.9%). Out of 244 patients, 8 ones (3.3%) experienced seizure. There was a significant correlation between seizure occurrence and family history of seizure (p=0.03) as well as fava bean consumption (p=0.019) as the causes of hemolysis; but not with infection as the cause of hemolysis, hemoglobin or hemoglobinuria level on admission, types of drinking water, place of living, and gender. Methemoglobinemia was considered as the main cause of the seizure. Although the rate of seizure was not so high (3.3%), it seems that seizure can be a critical and potentially life-threatening complication in these patients. Environmental factors may also play a role in the pathogenesis of the seizure in these patients.

Attention to the family care provider needs and their caring power is essential. Since mothers are considered as the child’s main care provider, this study aimed to determine the caring power and its predictors among mothers of children with cancer.
In this descriptive-correlational study, 196 mothers who had a child with cancer were selected through purposive sampling. The data were collected using two questionnaires, namely demographics questionnaire and the care power of the care providers of cancer patient questionnaire. The data were analyzed using SPSS 19 and running descriptive statistics and regression analysis.
The highest average score belonged to dimensions of “effective role play” (44.62 ± 5.28) and “trust” (14 ± 1.67), and the lowest ones respectively belonged to dimensions of “fatigue and resignation” (22.38 ± 6.33), “awareness” (8.46 ± 2.70), and “uncertainty” (12.38 ± 3.50). In addition, variables of educational level (p <0.001), adequacy of family income (p <0.001), and duration of illness (p0.29) were found as predictors of caring power.
The results of this study showed that the caring power of mothers with a child with cancer is favorable. High trust and effective role-play reduced fatigue and resignation of mothers, and low awareness about the provision of care caused uncertainty affecting negatively the care power. In addition, the adequacy of family income, the high level of mother's education, and the reduction in the duration of the disease had a direct impact on care power.

One of the most important phenotypic modifying factors for thalassemia is the presence of Xmn1 polymorphism. This retrospective study was performed to investigate the overall prevalence of Xmn1 polymorphism among Iranian β-thalassemia patients with homozygote IVSII-1mutation and to assess the relationship between Xmn1 polymorphism with patients’ hemoglobin levels and the response to hydroxyurea (Hu) therapy.
The present cross sectional study included 112 β-thalassemia patients with homozygote IVSII-1 mutation. Laboratory investigations included complete blood count and routine hematological indices were measured by Sysmex K1000 (Japan) blood auto analyzer.  To find the state of Xmn1 polymorphism, blood samples were collected from patients using EDTA containers for genomic DNA analysis. DNA extraction and amplification-refractory mutation to determine the Xmn-1 polymorphism were performed.
In total, 206 thalassemia patients including 112 patients diagnosed as thalassemia major and 94 patients diagnosed as thalassemia intermediate entered the study. The mean age at the start of transfusion was 5 ± 6.4 years old, and all of the patients received hydroxyurea. Twenty eight patients (14%) did not show any Xmn 1 polymorphisms (- / -), and 178 patients (86%) showed polymorphism either in one loci (- / +, 44 patients, 21.3%) or both loci   (+ / +, 134 patients, 65%).  Patients with Xmn1 polymorphism showed significantly higher age at diagnosis (p=0.002), higher age at start of transfusion (p=0.001), higher hemoglobin levels after treatment with hydroxyurea (p=0.005), and lower transfusion dependency (P=0.044).
The presence of Xmn1 polymorphism led to a delay in onset of blood transfusions, higher hemoglobin levels, better response to hydroxyurea treatment and milder phenotypic presentation among thalassemia patients with IVSII-1 mutation.

Childhood cancer (ChC) is very rare and occurs between birth and 14 years of age. There are several reports about ChC incidence from various regions of Iran, but with conflicting results.  The present study aimed to do a systematic review to estimate the accurate incidence rate of ChC among Iranian people.
This systematic review was performed based on the preferred reporting items for systematic reviews and meta-analyses (PRISMA) checklist in 2018. A literature search was conducted using international databases (Medline/PubMed, Scopus, ISI/Web of Knowledge, and Google Scholar) for English papers, and national databases (Scientific Information Database, MagIran, IranMedex, and IranDoc) for Persian papers which estimated the incidence rate of ChC in any geographical location in  Iran. The incidence rate of ChC was calculated using random-effect model.
Out of 157 papers in the primary searches, 12 studies were included by advanced screening and refinement. The crude incidence rate (CIR) of ChC in 0-14 years was 16.8 per 100,000 (95% CI: 9.04-24.56) for boys and 16.56 per 100,000 (95% CI: 10.51-22.62) for girls.
The incidence of ChC in Iran is higher compared to other parts of the world. Considering this issue, holding some interventional programs on tackling potential risk factors, including air pollution, in different regions of Iran is suggested.

Acute lymphoblastic leukemia is the most common malignancy in children with a 5-year survival rate, accounting for 80% of cases. Melanoma is rare in children and has been reported as a sporadically occurring secondary malignant neoplasm in children with acute lymphoblastic leukemia. This study presented a 10-year-old Iranian child with pre-B-cell acute lymphoblastic leukemia that was diagnosed at age 6. She was fully recovered after 2 years of treatment. One year and six months after cessation of treatment, she was referred with a 1×2 cm mass in her right parietal region of scalp. Biopsy of the lesion confirmed the diagnosis of malignant melanoma. Computed tomography scan of the chest and abdomen also confirmed extensive liver metastasis which was corroborated by liver biopsy. Bone scan also revealed bone metastases. Early diagnosis and treatment of these tumors is extremely important and these patients should be closely monitored and undergo regular physical examination.