Iranian Journal of Pediatric Hematology & Oncology

Global survival of pediatric patients with acute lymphoblastic leukemia from a Latin American Hospital

Sun, 30 Jun 2024 09:26:54 +0000

Background: Acute lymphoblastic leukemia (ALL) is the most common neoplasm in pediatric and adolescent populations. Overall survival has improved in recent decades. This study aimed to assess the overall survival of patients with pediatric ALL in a Latin American hospital.

Materials and Methods: A longitudinal and retrospective analytical study was conducted on 31 patients less than 16 years of age diagnosed with ALL at the hematology department of a Peruvian hospital during the period 2015-2016. Overall survival at 5 years was determined using the Kaplan-Meier curve with parametric log-rank tests, and the Cox regression model was employed to ascertain the hazard ratios of significant variables.

Results: The average age was 6 years, and 21 (67.7%) were female. The 5-year overall survival rate was 35%, with a median survival of 33 months (95% CI = 34.078-66.861). Being 10 years or older was associated with lower survival (p = 0.002). No significant association with B-cell acute lymphoblastic leukemia was found (p = 0.057).

Conclusion: The overall survival rate obtained was similar to that reported in other local studies; however, several international studies have reported better survival rates compared to our findings. Age was identified as a significant factor affecting survival.

Analysis of Pulmonary Complications in Pediatric Acute Lymphocytic Leukemia Patients following Three Years of Chemotherapy Treatment: A Cross-Sectional Study

Sun, 30 Jun 2024 09:34:30 +0000

Background: This study aimed to investigate the pulmonary side effects of chemotherapy drugs in children with Acute Lymphocytic Leukemia (ALL) three years after treatment. The results could be of great help in managing lung complications in pediatric oncology patients.

Materials and Methods: This cross-sectional descriptive study included 50 patients (22 males and 28 females) with ALL. Data were collected from patients' files, including age, sex, duration of illness, last dose of chemotherapy, and medications such as PEG.Asparginas, Cyclophosphamid, thioguanine, Dexamethason, cytarabin, cytosin.arabinosid, vincristine, mercaptopurine, and methotrexate. Pulmonary function tests (Forced Vital Capacity (FVC), FEV1 (Forced Expiratory Volume in the first second)) were assessed by spirometry.

Results: Out of 50 patients, 47 (94%) did not have pulmonary disorders, while 3 (6%) had pulmonary dysfunction. Forty-seven patients (94%) did not have respiratory symptoms. FVC results showed that 45 patients were normal, and 5 were abnormal. Similarly, 45 patients had normal FEV1, and 5 had abnormal results. Spirometry results were normal in 45 patients and abnormal in 5. A significant relationship was found between the use of these drugs in different doses and spirometry results, recurrence rate, and pulmonary complications (P <0.05). No significant relationship was observed between pulmonary dysfunction and other drugs (P> 0.05). No correlation was found between pulmonary complications due to chemotherapy with duration of chemotherapy, patient age, and patient gender (P> 0.05).

Conclusion: Pulmonary dysfunction and respiratory syndrome were observed in 6% of patients receiving chemotherapy. A significant relationship was found between the frequency of pulmonary and respiratory complications with some chemotherapy drugs in children with ALL. Further research is needed to optimize treatment strategies and minimize lung complications in pediatric oncology patients.

Analysis of how Serum Ferritin and the Aspartate Aminotransferase-to-Platelet Ratio Index (APRI) are correlated to Hepatic MRI T2* Findings in Children with Beta-Thalassemia Major

Roohollah Edalatkhah, Marjan Kargar, Maryamalsadat Yazdanparast — Sun, 30 Jun 2024 09:36:37 +0000

Background: Iron overload is a major complication in patients with beta-thalassemia major. Excessive iron accumulation leads to organ dysfunction. The regular assessment of iron is crucial to effectively manage iron overload in these patients. This study evaluated the correlation between serum ferritin levels, the Aspartate Aminotransferase-to-Platelet Ratio Index (APRI), and hepatic MRI T2* findings in children with beta-thalassemia major.

Materials and Methods: This retrospective analytical study was conducted at Shahid Sadoughi Hospital in Yazd in 2023. The research population comprised all the children under the age of 15 with beta-thalassemia major who had undergone multiple blood transfusions (at least ten units of blood).

Results: The participants in this study were 70 children with beta-thalassemia major, including 35 males and 35 females. Their mean age was 3.52 ± 10.76 years. The mean relaxation time of liver MRI T2* was 4.42 ± 4.91 ms. The participants also had the APRI score of 0.55 ± 1.90, the aspartate aminotransferase (AST) level of 23.82 ± 36.29, and the serum ferritin level of 285.01 ± 3244.04 (ng/mL). Based on MRI T2* results, 30% of the patients had a severe liver iron overload, 27.1% had a moderate overload, 21.4% had a severe overload, 18.6% had a mild overload, and 2.9% had near-normal iron levels.The AST level demonstrated a significant association with the type of chelation treatment (P = 0.003). The duration of blood transfusion (in years) showed a strong positive correlation with the patients' age (Pearson's coefficient = 0.996).

Conclusion: This study indicates the elevated serum ferritin levels and APRI scores in patients with beta-thalassemia major, most of whom have abnormal MRI T2* findings. However, no significant correlation was observed between the APRI score, serum ferritin level, and MRI T2* results.

A retrospective survey on follow-up of splenectomy patients due to β-thalassemia and Sickle cell Anemia in Karbala, Iraq during 2010-2023

Inas Muayad Mohammed Ali, Ashwaq Ali Hussein, Israa Mustafa Salih Al-Musawi — Sun, 30 Jun 2024 09:40:11 +0000

Background: Hemoglobinopathy is considered a common monogenetic genetic disorder worldwide. Splenectomy is considered a therapeutic strategy in patients with hemoglobinopathy. The aim of current study was to provide a survey on the splenectomy and 5 years follow-up in different clinical forms of β-thalassemia (intermedia, Major) and Sickle cell Anemia (SCA) patients who referred to Hereditary Blood Disease Center in the Karbala Teaching Hospital for Children in Karbala, Iraq.

Materials and Methods: In this retrospective study we tried to evaluate 126 hemoglobinopathy and thalassemia patients from Karbala City, Iraq. All cases of splenectomy due to hemoglobinopathy and thalassemia during 2010-2023 who referred to the Hereditary Blood Disease Center in the Karbala Teaching Hospital for Children in Karbala, Iraq were included. Patient data was collected at three-time points. The first was after the splenectomy, the second during 1-5 years, and the third step after 5 years. Clinical and laboratory data were retrieved from the patient’s file.

Results: The mean age of splenectomy of included patients was 14.1±7.5 years. From 126 cases, 103 (81.74%) were β-Thalassemia, 13 (10.32%) were SCA, and 10 (7.94%) were Sickle cell beta-thalassemia. The mean age in SCA was significantly less than two other groups (mean age in β-Thalassemia, SCA and Sickle cell beta thalassemia were 18.2±8.7, 24.2±12.7 and 25.2±9.5, respectively) (p=0.008). Platelet and WBC count represents a significant increase during 1-5 years after splenectomy in comparison with 1 year after splenectomy (for Platelet and WBC p=0.03 and 0.001, respectively).

Conclusion: splenectomy is considered the last therapeutic option in hemoglobinopathy patients. All Hemoglobinopathy patients represented significant improvement after splenectomy. Because there was no suitable treatment in the past, splenectomy was considered a therapeutic solution. It should be said that periodic follow-up of splenectomy patients in hemoglobinopathy plays an important role in improving the management of these diseases.

In silico analysis of genes and molecular pathways involved in the pathogenesis of follicular lymphoma

Sun, 30 Jun 2024 10:49:52 +0000

Background: Follicular lymphoma (FL) is a common form of non-Hodgkin lymphoma, characterized by abnormal B-cell growth within the germinal center. Research has shown the role of genes and molecular pathways in the pathogenesis of FL. However, the main factor of pathogenesis has not been determined. Therefore, in this study, the genes and molecular pathways related to the pathogenesis of FL were evaluated using a systems biology approach.

Materials and Methods: In this study (bioinformatics analysis), the GSE32018 database was used for data analysis. This database was extracted from Gene Expression Omnibus (GEO). The sample of this database was 36, which included normal and FL samples. For this purpose, 23 cases were FL and 13 were healthy samples. Protein-protein interaction (PPI) is performed to show the interaction between DEGs. STRING software is used for this purpose. Associations between the hub genes, transcription factors, and microRNAs were assessed using the miRTarBase and TRRUST databases. The criteria used for data analysis included log fold change greater than one and p < 0.05.

Results: After evaluating and analyzing the data, the results showed that 866 DEGs were identified between the control and FL samples. Of this population, 231 cases of UP regulation and 635 cases of downregulation were in FL samples compared to control samples. PPI network and hub gene analyses identified 7 hub genes, including RPL37A, MRPS7, RPS14, RPS28, RPL34, RPS20, and RPS3. According to the results, hsa-miR-191-5p has the highest interactions with hub genes among miRNAs, and KDM5A has the most interactions among TFs.

Conclusion: Identifying genes and molecular pathways can be effective in designing therapeutic strategies and preventing the proliferation of FL cells, thereby increasing patients’ survival.

Evaluating the expression of LKB1, SHMT1, and GLDC genes in Acute Lymphoblastic Leukemia patients

Sun, 30 Jun 2024 11:24:17 +0000

Background: Epigenetic changes in cancer cells have an immense effect on tumorigenesis. As a tumor suppressor and an epigenetic regulator, liver kinase B1 (LKB1) reduces gene methylation by downregulating metabolic pathways such as the serine-glycine pathway. This study seeks to examine the gene expression levels of serine hydroxymethyltransferase 1 (SHMT1) and glycine decarboxylase (GLDC), as two serine-glycine pathway regulatory genes, along with LKB1 in acute lymphoblastic leukemia (ALL) patients.

Materials and Methods: In this analytical study, qRT-PCR was used to evaluate the gene expression levels of LKB1, SHMT1, and GLDC in 50 ALL patients with an average age of 11.64 ± 10.6 years. The patients were compared to 10 healthy controls. Subsequently, the correlation between the gene expression levels and the patients’ demographic data was investigated.

Results: No significant difference was found between the ALL patients and the control individuals in terms of LKB1 and GLDC gene expressions, but SHMT1 was significantly overexpressed in the ALL patients (p = 0.003). Moreover, there was a significant association between GLDC and the other SHMT1 (p = 0.020) and LKB1 (p = 0.047). No significant connection was also found between the age (pL = 0.304, pS = 0.305, pG = 0.899), gender (pL = 0.475, pS = 0.299, pG = 0.388), and blast percentage (pL = 0.335, pS = 0.148, pG = 0.459) of the patients and the genes.

Conclusion: The increased expression of SHMT1 suggests the oncogenic role of this gene. Thus, the present study offers a novel diagnostic marker in ALL patients.

Analysis and Identification of Rare and Prevalent Breakpoints in Chromosomal rearrangements in Adult and Pediatric with B-Acute Lymphoblastic Leukemia (B-ALL): A Systematic Review

Roghayeh Shahshahani, Mehri Khatami, Mohammad Mehdi Heidari, Parisa Naji — Sun, 30 Jun 2024 11:27:18 +0000

Background: B-cell acute lymphoblastic leukemia (B-ALL) is a complex disorder that includes multiple genetic changes, one of the main causes of which is rare and common chromosomal translocations that lead to abnormal gene fusions. This abnormal fusion produces a new protein that causes the leukemia cells. These types of rearrangements usually occur in lymphoma and result in the movement of genetic material between different chromosomes or within chromosomes. This systematic review aims to evaluate published studies and investigate the role and importance of common and rare chromosomal translocations in the occurrence of B-ALL.

Material and Methods: This systematic review investigated and evaluated the evidence regarding the effect of chromosomal translocations in adults and pediatrics with B-ALL. This review was based on the preferred reporting items for systematic reviews and meta-analysis checklists. A literature search was conducted using international databases (such as PubMed, Web of Science, Scopus, Research Gate, Google Scholar, and Cochrane Systematic Reviews database). Only English-language articles published between January 2010 and January 2023 including MESH terms such as ALL, B-ALL, and chromosomal translocations were selected. Seventy-five related studies had the necessary criteria to be examined in the present study.

Results: A total of 237 articles were retrieved in the online search. The excluded articles included research on other types of leukemia in adults and children, descriptive studies, case studies, studies related to animal models of leukemogenesis, and comparisons of common treatment methods of leukemia grouping cancers. Finally, seventy-five studies were identified as eligible for inclusion in this systematic review.

Conclusion: This review provides a comprehensive assessment of common and rare chromosomal translocations that can lead to the development of cancer cells. Chromosomal translocations play a role as diagnostic markers and important prognostic indicators and help specialists adjust treatment approaches according to their occurrence.

Pediatric Myelofibrosis: A Rare Entity Posing a Diagnostic Challenge

Vandana Puri, Kusha Sharma, Sunita Sharma, Shailaja Shukla, Nupur Parakh — Sun, 30 Jun 2024 11:30:33 +0000

Myeloproliferative neoplasms are clonal hematopoietic stem cell disorders showing proliferation of one or more myeloid lineages. These disorders are characterized by Janus Kinase 2 (JAK2 V617F), Myeloproliferative leukemia (MPL), and Calreticulin (CALR) gene mutations and are seen more commonly in the elderly. These pathognomonic mutations are often absent in children and hence pose a diagnostic challenge. The entire onus of correct diagnosis relies heavily on detailed clinical and laboratory investigations. In the present work, we discuss 4 cases of pediatric myelofibrosis, three of which were secondary to hematolymphoid malignancies, while the remaining one had primary myelofibrosis. Pediatric primary myelofibrosis is a rare entity in children and quite different from adult primary myelofibrosis. The cases in our study show transformation into acute myeloid leukemia, which is associated with an adverse prognosis. Given the rarity of myelofibrosis in children, early and correct diagnosis helps in timely initiation of treatment, which may favorably alter the prognosis.