Iranian Journal of Pediatric Hematology and Oncology
Volume:10 Issue: 3, Summer 2020

Acute leukemias comprise a heterogeneous group of diseases characterized by rapid and uncontrolled clonal expansion of progenitor cells of the hematopoietic system. Immunophenotyping helps subclassify acute leukemias into subgroups with prognostic implications.

This descriptive observational cross-sectional study was performed on 80 newly diagnosed cases of acute leukemia in children up to 18 years of age from August 2012 to Febuary 2014.
The immunophenotypic analysis was performed by Beckman Coulter Flow cytometer using monoclonal antibodies against various cell surface / cytoplasmic antigens.

Out of 80 cases, 68 cases had acute lymphocytic leukemia (ALL) and 12 cases had acute myelocytic leukemia (AML). In this study, 68 cases of ALL could be categorized into 53 cases of B-ALL, 13 of T-ALL, and 2 cases of Bi-phenotypic acute leukemia (BAL). Twelve cases of AML comprised of 7 cases of AML with minimal differentiation; 2 of AML with cases of AML with maturation, and 3 cases of acute myelomonocytic leukemia. Hepatosplenomegaly was seen in the majority of cases in both ALL and AML. Most cases had a total leucocyte count between 10,000 and 50,000/µl and platelets <100,000/µl.  Hemoglobin levels were < 7.5 g/dl in the majority of them. CD19 and CD79a were 100 % sensitive for B ALL, while cCD3 was 100% sensitive for T-ALL. MPO was positive in all cases of AML.

Immunophenotyping must be done in all AL cases as it helps in risk-stratification and follow up of patients for evaluating minimal residual disease.

Given that few studies regarding the effect of Aloe vera (A. vera) on prevention of fever and neutropenia on acute lymphoid leukemia (ALL) disease, the aim was to evaluate the effect of A.vera on prevention of fever and neutropenia in children with ALL.

This randomized clinical trial study was conducted on 60 children with mean age 5.6± 2.9 years in Oncology department of Shahid Sadoughi hospital during 2018-2019. All these children were underwent chemotherapy. Then, the patients were randomly classified into two groups. The first group received 10 ml A. vera syrup (0.5 mg/ml) for 30 days (case group) and the second group did not receive A. vera syrup (control group). Complete blood count (CBC) tests and frequency of fever, infection, neutropenia, and hospitalization were evaluated in case and control groups before and after intervention. Wilcoxon, T test, and Chi Square test were used for data analysis.

Significant reduction was observed in case group in comparison with control group regarding the frequency of fever (0.19 ±0.107 vs 1.07±0.206), infection (0.81 ±0.2 vs 1.87± 0.236), hospitalization (0.37±0.178 vs 1.33 ±0.187), and neutropenia (0.11± 0.062 vs 0.80 ± 0.2) (p<0.01). Moreover, significant increase was observed in case group in comparison with control group considering neutrophil count (2597.5±243.1 vs 1106.53±161.6) and white blood cell (WBC) (4062.96 ± 276.6 vs 2566.67 ±175.5) (p<0.01). In addition, recovery of appetite was significantly greater in case group than control group (p=0.023). Furthermore, significant difference was observed in case group before and after intervention regarding these parameters (p<0.05).

According to findings, A. vera consumption decreased frequency of infection, neutropenia, fever, and hospitalization and increased WBC and neutrophil count.  Moreover, appetite was improved in these patients. Therefore, it seems that A.vera can be used for improving quality of life in children with ALL

Decitabine (5-aza-2'-deoxycytidine, DAC) is a deoxycytidine analog currently used as an effective drug against myelodysplastic syndromes and acute myeloid leukemia. Although various studies have pointed out the epigenetic effects of this drug, its epigenetic mechanisms in different leukemic cell lines are not specified. In this lab trial study, possible epigenetic effects of decitabine on leukemia cell lines Hl-60 (acute promyelocytic leukemia) and Nalm-6 (acute pre-B cell lymphoblastic leukemia) vs. normal peripheral blood mononuclear cells (PBMCs) are compared.

At the logarithmic phase of growth, the cultured cells Hl-60 and Nalm-6 obtained from Tehran Pasteur Institute, Iran, were treated for 24 hr with 1 μM of decitabine, a dose selected from literature and the MTT viability assay. Normal PBMCs were obtained from a pool of 3 healthy adult volunteer males, and cultured simultaneously in the same manner. The gene expressions of epigenetic enzymes DNA methyltransferases (DNMTs) were assessed with the real-time PCR technique before and after treatment. The GAPDH gene expression served as the calibrator, while normal PBMCs were used for comparison.

The expressions of DNMT1 and DNMT3B in lymphoblasts were significantly (p=0.0017 and p=0.0489, respectively) decreased after treatment with decitabine, while the expression of DNMT3A was significantly (p=0.0022) increased. In leukemic promyelocytes the expressions of DNMT1 and DNMT3B in lymphoblasts were significantly (p=0.0222 and p=0.0452, respectively) decreased after treatment with decitabine, while the expression of DNMT3A was significantly (p=0.0013) increased.

One of the mechanisms by decitabine to inhibit the proliferation of both myeloid and lymphoid acute leukemia cells maybe by altering the gene expression of DNMTs.

MicroRNAs (miRNAs) are noncoding RNAs that control the expression of their target mRNAs. It affects cancer cell proliferation and apoptosis as oncogenes or tumor suppressors. Dysregulation of miRNAs expression leads to the development of various cancers. Therefore, for the first time in this field, this study investigated the effect of overexpression of microRNA-630 on the Jurkat cells.

: In this experimental study, the Jurkat cells were divided into the four groups, i.e. non-transfected control group (A), scramble (B), transfected with 50 nM concentrations of miR-630 (C), and treated with 100 nM miR-630 (D). MiR-630 transfection was performed by lipofectamine 2000. Cancer cell growth in each group was analyzed with MTT assay. Flow cytometry investigated percent of viable, necrotic, and apoptotic cells. Quantitative real-time reverse transcription polymerase chain reaction (qRT-PCR) measured the expression of P53, P21, and BCL2 genes. SPSS (version 21) especially Kruskal-Wallis and Mann-Whitney U tests were utilized for data analysis.

The results of MTT assay showed that the cell growth rates in C (118%) and D (136%) groups were significantly higher than that in the control group (P= 0.037 vs. 0.034). The percentage of early and late apoptosis in C (3.1% P=0.01, 4.2% P=0.02) and D (0.5% P=0.008, 0.4% P=0.006) groups were significantly lower than those in the control group. The expression of p53 and p21 in C (0.7 P=0.037, 0.62 P=0.034) and D (0.44 P=0.034, 0.53 P=0.038) Groups were significantly decreased compared with the control group. The expression of B-cell lymphoma-2 (Bcl2) in C (1.85) and D (3.26) groups were significantly increased compared with the control group (P= 0.037 vs. 0.024).

Overexpression of miR-630 led induction of T-ALL cell growth and reduction of their apoptosis. These results emphasized that miR-630 contributed as an oncogenic microRNA in T-ALL cells.

Lumbar puncture (LP) is a worth procedure in diagnosis of oncological diseases and intrathecal administration of antineoplastic drugs. The effort should be to minimize pain of LP in children with cancers. This clinical trial was done to compare success rates in performing LP and reducing anxiety and pain of LP in sitting and lateral decubitus positions in 1 to 5-year-old children.

In a not-blinded clinical trial, 80 children aged 1-5 years, undergoing LP in Pediatric Ward of Shahid Sadoughi Hospital, Yazd, Iran, from May to September 2019, were randomly allocated to two groups. Intravenous 0.5mg/kg midazolam was injected in all patients five minutes before LP, and LP was performed in sitting position in group I and in lateral decubitus position in group II. Primary outcomes included rate of successful LP, anxiety and pain scores before LP and during needle insertion to skin for LP, and secondary outcomes comprised of success rates in decrease of anxiety (anxiety score of four and more) and pain (pain score of less than three) when the needle was inserted to skin for LP.

Thirty-eight girls and 42 boys with the mean age of 2.51 ± 0.32 years were evaluated. Success rates in performing LP (70 % in sitting vs. 65% in decubitus position, P=0.5), decrease of LP anxiety (77 % in sitting vs. 75% in decubitus groups, P=0.8) and reduction of pain during skin needle insertion for LP (72 % in sitting vs. 67% in decubitus position, P=0.7) were not significantly different between the two positions.

Rates of success in performing LP and reduction of its pain and anxiety in children were equal in lateral decubitus and sitting posi tions and, in 1 to 5-year-old sick children with or without cardiorespiratory difficulties, LP can be done in lateral decubitus or sitting position.

Cancer is one of the main causes of mortality and disability worldwide, especially in developing countries. The most prevalent type of cancer among children is acute lymphoblastic leukemia (ALL). Given the increasing rates of ALL in children and the financial burden, this study aimed to investigate the role of the budget provided by non-governmental organizations (NGOs) in rising life expectancy in children with cancer.

This research was a descriptive-analytical study and performed on 30 children with ALL in Mohammad Rasool Allah charity (MAHAK branch) during 2017-2019. The Schneider life expectancy questionnaire was used to collect information. Data were completed by interviewing of children before and after accepting in charitable foundation.

In current study, there was significant difference between the mean score of Schneider life expectancy before and after intervention (p <0.01). In addition, assessment of life expectancy in individuals showed that after entering of participants to study, items such as "in my opinion, there are many ways to achieve the things that are important in my life”, “I think there are many ways to get rid of the pressures and” there are many ways to solve the problem with score 135, 130 and 120 were higher than other items.

According to results of this study, it seems that the support of NGOs can improve life expectancy of children with cancer. Therefore, it is proposed that the role of NGOs is considered more than ever

MicroRNAs (miRNAs, miRs) are small endogenous non-coding RNAs that regulate the expression of protein-encoding genes at the post-transcriptional level. Several studies have described the role of miRNAs in T-cell acute lymphoblastic leukemia (T-ALL), including tumor suppressor and oncogenic miRNAs. Down-regulation of miRNA expression is a prominent feature of human malignancy. This down-regulation can be triggered by certain chromosomal rearrangements, such as somatic deletions or translocations. New functional studies showed that dysregulation of microRNAs plays significant roles in cellular proliferation, differentiation, apoptosis, in human cancers such as leukemia. In this review, we focused on the major recent findings in the microRNA signatures in ALL pathogenesis and we discussed the potential use of cellular and circulating miRNAs as new molecular biomarkers for diagnosis and prognosis of acute lymphoblastic leukemia. In this systematic review article, 76 articles were collected from 2004 to February 2019. We chose research and review articles from open access journals which include MeSH terms such as ALL, T-ALL, microRNA, oncogenic miRNA, Tumor Suppressor miRNA, microRNA Expression and signaling pathway. Investigation of data showed that alterations in oncogenic and tumor suppressor miRNAs expression changed the expression of genes related to accurate cell functions and consequently the pathogenicity of T-ALL.

There are more than 400 different variations on α-globin protein, and most of them are not associated with noticeable clinical manifestation. Hemoglobin (Hb) is an oxygen-transporting protein and Hb Daneshgah- Tehran is an α-globin variant that for the first time was reported from Iran in a case with normal haematological indices. The capillary electrophoresis of an 8-year- old-girl with normal hematological parameters showed a peak in the location of Hb S (19.2%) with small amount of Hb A2 variant. The sequencing analysis indicated that the patient was heterozygote for Hb Daneshgah- Tehran (HBA1:c.218A>G p.His72Arg). Alpha and beta thalassemia are common health problems in north of Iran, and about 15% of Mazandarani people are carriers for alpha globin gene deletions, hence premarital screening program can help diagnosis of common and rare hemoglobinopathies. This case was the first report on Hb Daneshgah- Tehran from Mazandaran and the second one from Iran. The presented case showed that Hb Daneshgah- Tehran had haematological indices in normal range, and for the detection of this Hb variant, electrophoresis and PCR sequencing methods should be applied.