Background: The mainstay of managing severe β-thalassemia remains lifelong blood transfusion. Mismatched red blood cell phenotypes between donors and recipients in multiple blood transfusions can result in the development of alloimmunization in recipients. The aim of this study was to determine the frequency of major and subgroup antigens and their phenotypes in thalassemia major patients.
Materials and Methods: This cross-sectional descriptive study was performed on 105 patients with thalassemia major who referred to Baghaei Hospital in Ahvaz in 2021. Their alloimmunization to erythrocyte antigens was determined with standard tubular antibody search kits.
Results: Among the thalassemia major patients participating in the study, 51 were female (48.45%). The mean age of the participants was 21.10 ± 5.8 years. Out of the 105 patients studied, 26 had detectable alloantibodies in the serum (24.7%). The two groups of patients with positive and negative alloantibodies were significantly different in terms of Rh and C blood groups (P-values of 0.03 and 0.05, respectively). There was no significant association between the existence alloantibody and age, gender, spleen condition and the time of first transfusion (P > 0.05).
Conclusion: It was concluded that red blood cell matching, at least for Rh and C groups, is necessary to prevent alloimmunization in thalassemia major patients.
{"title":"Frequency of Red Cell Alloimmunization in Patients with Thalassemia Major: A Report from the Southwest of Iran","authors":"Bijan Keikhaei, Homayoun Yousefi, Arash Alghasi, Asaad Sharhani, Roya Khazaei","doi":"10.18502/ijpho.v13i4.13772","DOIUrl":"https://doi.org/10.18502/ijpho.v13i4.13772","url":null,"abstract":"Background: The mainstay of managing severe β-thalassemia remains lifelong blood transfusion. Mismatched red blood cell phenotypes between donors and recipients in multiple blood transfusions can result in the development of alloimmunization in recipients. The aim of this study was to determine the frequency of major and subgroup antigens and their phenotypes in thalassemia major patients.
 Materials and Methods: This cross-sectional descriptive study was performed on 105 patients with thalassemia major who referred to Baghaei Hospital in Ahvaz in 2021. Their alloimmunization to erythrocyte antigens was determined with standard tubular antibody search kits.
 Results: Among the thalassemia major patients participating in the study, 51 were female (48.45%). The mean age of the participants was 21.10 ± 5.8 years. Out of the 105 patients studied, 26 had detectable alloantibodies in the serum (24.7%). The two groups of patients with positive and negative alloantibodies were significantly different in terms of Rh and C blood groups (P-values of 0.03 and 0.05, respectively). There was no significant association between the existence alloantibody and age, gender, spleen condition and the time of first transfusion (P > 0.05).
 Conclusion: It was concluded that red blood cell matching, at least for Rh and C groups, is necessary to prevent alloimmunization in thalassemia major patients.","PeriodicalId":44212,"journal":{"name":"Iranian Journal of Pediatric Hematology and Oncology","volume":"47 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135198132","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-10-08DOI: 10.18502/ijpho.v13i4.13768
Shiva Rahbar Yazdi, Mohammad Hosein Zare, Mohammad Ali Broomand
Background: The scattered radiation from the treatment volume might be more significant for children than for adults because of life expectancy. The present study used biological effects of ionizing radiation (BEIR) VII models to estimate radiation-induced secondary cancer risks in irradiated organs following three-dimensional conformal radiation therapy (3D-CRT) of Acute Lymphocytic Leukemia (ALL) in children. Both excess absolute risk (EAR) and excess relative risk (ERR) models were used to estimate the secondary cancer risks of eye lenses, thyroid, parotid, breast, and region overlying ovaries.
Materials and Methods: In this expository cross-sectional study, from 45 patients who were examined, 16 patients age under 18 years (mean age of 9.6) met the criteria for entering the study in Shahid Ramezanzadeh Hospital in Yazd underwent whole brain radiotherapy (WBRT) using COMPACT accelerator. Measurement was performed using thermoluminescent dosimeters (TLD). After radiation therapy, the secondary cancer risk in these organs was calculated.
Results: The organ dose mean values in female patients were 1.8±0.1, 1.65±0.61, 1.47±0.04, 0.1±0.03, and 1.58±0.52 cGy in the eye lenses, parotid, thyroid, breast, and region overlying ovaries, respectively and 2.7±0.6, 0.76±0.17, 0.6±0.05, and 0.005±0.002 cGy for eye lens, parotid, thyroid, breast, and testis of male patient, respectively. The ERR and EAR were estimated after 3, 5, 10, 15 and 20 years for eye lens, parotid breast, and ovary/testis for female/male.
Conclusion: Higher risk values were found for eye lenses and thyroid. The scattered rays decreased by increasing the organ distance from the treatment radiation field.
{"title":"Estimation of Secondary Cancer Risk of Radiosensitive Organs for Leukemia from Head Radiotherapy in Pediatric Patients","authors":"Shiva Rahbar Yazdi, Mohammad Hosein Zare, Mohammad Ali Broomand","doi":"10.18502/ijpho.v13i4.13768","DOIUrl":"https://doi.org/10.18502/ijpho.v13i4.13768","url":null,"abstract":"Background: The scattered radiation from the treatment volume might be more significant for children than for adults because of life expectancy. The present study used biological effects of ionizing radiation (BEIR) VII models to estimate radiation-induced secondary cancer risks in irradiated organs following three-dimensional conformal radiation therapy (3D-CRT) of Acute Lymphocytic Leukemia (ALL) in children. Both excess absolute risk (EAR) and excess relative risk (ERR) models were used to estimate the secondary cancer risks of eye lenses, thyroid, parotid, breast, and region overlying ovaries.
 Materials and Methods: In this expository cross-sectional study, from 45 patients who were examined, 16 patients age under 18 years (mean age of 9.6) met the criteria for entering the study in Shahid Ramezanzadeh Hospital in Yazd underwent whole brain radiotherapy (WBRT) using COMPACT accelerator. Measurement was performed using thermoluminescent dosimeters (TLD). After radiation therapy, the secondary cancer risk in these organs was calculated.
 Results: The organ dose mean values in female patients were 1.8±0.1, 1.65±0.61, 1.47±0.04, 0.1±0.03, and 1.58±0.52 cGy in the eye lenses, parotid, thyroid, breast, and region overlying ovaries, respectively and 2.7±0.6, 0.76±0.17, 0.6±0.05, and 0.005±0.002 cGy for eye lens, parotid, thyroid, breast, and testis of male patient, respectively. The ERR and EAR were estimated after 3, 5, 10, 15 and 20 years for eye lens, parotid breast, and ovary/testis for female/male.
 Conclusion: Higher risk values were found for eye lenses and thyroid. The scattered rays decreased by increasing the organ distance from the treatment radiation field.","PeriodicalId":44212,"journal":{"name":"Iranian Journal of Pediatric Hematology and Oncology","volume":"88 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135198294","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-10-08DOI: 10.18502/ijpho.v13i4.13769
Farshad Heidari, Mohammad Faranoush, Ali Amini, Elahe Rahimian, Kamyar Kazemi, Mostafa Paridar, Majid Safa
Background: Despite breakthroughs in the development of chemotherapy drugs to treat pediatric B-acute lymphoblastic leukemia (B-ALL), the relapse rate remains a major therapeutic challenge, requiring more detailed characterization of molecular elements underlying disease development and resistance to treatment. Checkpoint kinase 1 (CHK1) and checkpoint kinase 2 (CHK2) are two critical mediators of the DNA damage response (DDR) mechanism that activate the downstream components responsible for DNA repair, cell cycle regulation, and apoptosis. It has been shown that altered expression of CHK1 and CHK2 in various tumor entities promotes tumorigenesis and disease progression.
Materials and Methods: In this case-control study, we evaluated the relative expression status of CHK1 and CHK2 genes in pediatric B-ALL patients at diagnosis (n=20), during complete remission (n=23) and relapse phase (n=10), as well as 20 peripheral blood samples from healthy children as a normal control group. The mRNA expression levels of CHK1 and CHK2 were determined by the Real-time PCR method. Data were compared using the Mann–Whitney U test for the relative expression level of target mRNA in different phases of B-ALL. Data were presented as median and statistical significance was described as a P-value less than 0.05.
Results: Our results revealed that CHK1 expression increased in newly diagnosed patients than in healthy individuals (p ≤ 0.001). Relapsed patients had higher CHK1 expression than the newly diagnosed (p ≤ 0.05) and complete remission (p ≤ 0.001) counterparts. CHK2 was overexpressed in all phases of the diseases (p ≤ 0.001) without any significant alteration among the studied groups.
Conclusion: Given the CHK1 ability to endow cancer cells with a survival advantage upon chemotherapy, the present study suggests it as a potentially promising target in the fight against B-ALL.
{"title":"Overexpression of CHK1 and CHK2 in pediatric patients of B-acute lymphoblastic leukemia","authors":"Farshad Heidari, Mohammad Faranoush, Ali Amini, Elahe Rahimian, Kamyar Kazemi, Mostafa Paridar, Majid Safa","doi":"10.18502/ijpho.v13i4.13769","DOIUrl":"https://doi.org/10.18502/ijpho.v13i4.13769","url":null,"abstract":"Background: Despite breakthroughs in the development of chemotherapy drugs to treat pediatric B-acute lymphoblastic leukemia (B-ALL), the relapse rate remains a major therapeutic challenge, requiring more detailed characterization of molecular elements underlying disease development and resistance to treatment. Checkpoint kinase 1 (CHK1) and checkpoint kinase 2 (CHK2) are two critical mediators of the DNA damage response (DDR) mechanism that activate the downstream components responsible for DNA repair, cell cycle regulation, and apoptosis. It has been shown that altered expression of CHK1 and CHK2 in various tumor entities promotes tumorigenesis and disease progression.
 Materials and Methods: In this case-control study, we evaluated the relative expression status of CHK1 and CHK2 genes in pediatric B-ALL patients at diagnosis (n=20), during complete remission (n=23) and relapse phase (n=10), as well as 20 peripheral blood samples from healthy children as a normal control group. The mRNA expression levels of CHK1 and CHK2 were determined by the Real-time PCR method. Data were compared using the Mann–Whitney U test for the relative expression level of target mRNA in different phases of B-ALL. Data were presented as median and statistical significance was described as a P-value less than 0.05.
 Results: Our results revealed that CHK1 expression increased in newly diagnosed patients than in healthy individuals (p ≤ 0.001). Relapsed patients had higher CHK1 expression than the newly diagnosed (p ≤ 0.05) and complete remission (p ≤ 0.001) counterparts. CHK2 was overexpressed in all phases of the diseases (p ≤ 0.001) without any significant alteration among the studied groups.
 Conclusion: Given the CHK1 ability to endow cancer cells with a survival advantage upon chemotherapy, the present study suggests it as a potentially promising target in the fight against B-ALL.","PeriodicalId":44212,"journal":{"name":"Iranian Journal of Pediatric Hematology and Oncology","volume":"22 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135198617","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-10-08DOI: 10.18502/ijpho.v13i4.13771
Marwan S. Al-Nimer, Raz M. Hamasalih, Rawa Ratha
Background: Iron chelating agents (ICAs) may induce changes in the blood and the liver indices. This study aimed to compare the effects of deferasirox (oral) and deferoxamine (parenteral) on the hematological and liver indices.
Materials and Methods: A cross-sectional study was conducted on patients at the Thalassemia Center in Sulaymaniyah, Iraq. The study included 76 β-thalassemia major children (37 females and 39 males, with a median age of 6 years). The patients were divided into Group I (n = 51, treated with deferasirox) and Group II (n = 25, treated with deferoxamine). Complete blood count and liver enzymes (alanine [ALT] and aspartate [AST] aminotransferase) were determined; the hemoglobin densities were calculated to differentiate absolute from restrictive iron deficiency; and the fibrosis-4 score (FIB-4, aspartate-to-platelet ratio index (APRI), and (AST/ALT ratio) were calculated.
Results: Hemoglobin density indices showed restricted iron deficiency in both treated groups. However, serum ferritin level was higher in Group II than in Group I (1.9 times higher, p=0.037).
Also, the median value of MCV in Group II was significantly higher than in Group I (79.8 fL vs. 77.0 fL, respectively). In contrast, liver fibrosis indices defined with the mean values of AST-to-ALT ratio and FIB-4 score were higher in Group I compared to Group II. A positive and significant correlation was observed between APRI level and serum ferritin in Group I (r = 0.518, df = 49, p<0.001).
Conclusions: Based on the data, it can be concluded that both deferasirox and deferoxamine affect red blood cells parameters, which may be related to their function as ICAs, leaing to temporary iron deficiency in treated patients. Both drugs may induce inconsistent changes in the liver which are highly associated with circulating ferritin level. However, the destructive effect of deferasirox on the liver is more evident, leading to the induction of fibrosis
{"title":"Effects of deferoxamine versus deferasirox on hematology and liver parameters in children with beta-thalassemia major: A cross-sectional study from a single center","authors":"Marwan S. Al-Nimer, Raz M. Hamasalih, Rawa Ratha","doi":"10.18502/ijpho.v13i4.13771","DOIUrl":"https://doi.org/10.18502/ijpho.v13i4.13771","url":null,"abstract":"Background: Iron chelating agents (ICAs) may induce changes in the blood and the liver indices. This study aimed to compare the effects of deferasirox (oral) and deferoxamine (parenteral) on the hematological and liver indices.
 Materials and Methods: A cross-sectional study was conducted on patients at the Thalassemia Center in Sulaymaniyah, Iraq. The study included 76 β-thalassemia major children (37 females and 39 males, with a median age of 6 years). The patients were divided into Group I (n = 51, treated with deferasirox) and Group II (n = 25, treated with deferoxamine). Complete blood count and liver enzymes (alanine [ALT] and aspartate [AST] aminotransferase) were determined; the hemoglobin densities were calculated to differentiate absolute from restrictive iron deficiency; and the fibrosis-4 score (FIB-4, aspartate-to-platelet ratio index (APRI), and (AST/ALT ratio) were calculated.
 Results: Hemoglobin density indices showed restricted iron deficiency in both treated groups. However, serum ferritin level was higher in Group II than in Group I (1.9 times higher, p=0.037).
 Also, the median value of MCV in Group II was significantly higher than in Group I (79.8 fL vs. 77.0 fL, respectively). In contrast, liver fibrosis indices defined with the mean values of AST-to-ALT ratio and FIB-4 score were higher in Group I compared to Group II. A positive and significant correlation was observed between APRI level and serum ferritin in Group I (r = 0.518, df = 49, p<0.001).
 Conclusions: Based on the data, it can be concluded that both deferasirox and deferoxamine affect red blood cells parameters, which may be related to their function as ICAs, leaing to temporary iron deficiency in treated patients. Both drugs may induce inconsistent changes in the liver which are highly associated with circulating ferritin level. However, the destructive effect of deferasirox on the liver is more evident, leading to the induction of fibrosis","PeriodicalId":44212,"journal":{"name":"Iranian Journal of Pediatric Hematology and Oncology","volume":"88 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135197990","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-10-08DOI: 10.18502/ijpho.v13i4.13774
Pranab Kumar Dey, Eshita Das, Nairit De
Hepatitis A Virus (HAV) infection is a benign, self-limited gastrointestinal infection of children. Autoimmune hematological manifestation is very rare in children. Here, we report an 11-year-old male child having HAV infection with acute liver failure, complicated with persistent thrombocytopenia and haematuria during the course of illness and eventually diagnosed as a case of HAV infection associated with immune thrombocytopenic purpura. The child was treated successfully with a short course of steroid therapy.
{"title":"Immune Thrombocytopenic Purpura: An uncommon manifestation of Hepatitis A with acute liver failure","authors":"Pranab Kumar Dey, Eshita Das, Nairit De","doi":"10.18502/ijpho.v13i4.13774","DOIUrl":"https://doi.org/10.18502/ijpho.v13i4.13774","url":null,"abstract":"
 
 
 
 
 
 
 
 
 
 
 Hepatitis A Virus (HAV) infection is a benign, self-limited gastrointestinal infection of children. Autoimmune hematological manifestation is very rare in children. Here, we report an 11-year-old male child having HAV infection with acute liver failure, complicated with persistent thrombocytopenia and haematuria during the course of illness and eventually diagnosed as a case of HAV infection associated with immune thrombocytopenic purpura. The child was treated successfully with a short course of steroid therapy.
 
 
 
 
 
 
 
 
 
 
 
 
 
","PeriodicalId":44212,"journal":{"name":"Iranian Journal of Pediatric Hematology and Oncology","volume":"32 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135198102","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-10-08DOI: 10.18502/ijpho.v13i4.13770
Azin Elmi, Amir Atashi, Nematollah Gheibi, Shahin Amiri, Monireh Ajami, Mansoureh Ajami, Razieh Mohammadihaji, Naeimeh Khodabandeloo, Mehdi Azad
Background: Human bone marrow mesenchymal stem cells (hBM-MSCs), as supporters for hematopoiesis, differentiate into osteoblasts and adipocytes. Studies showed that infection of hBM-MSCs by Parvovirus B19 (B19V) can affect the differentiation capability of hBM-MSCs. This study aims to evaluate B19V effects on the differentiation of hBM-MSCs.
Materials and Methods: In this experimental study hBM-MSCs were cultured up to passage 3. Nucleofection was subsequently employed to deliver a plasmid containing the B19V genome into the cells. The transfected cells were then differentiated into osteoblast and adipocyte lineages. qRT-PCR was then performed to analyze the differentiation 14 days after transfection.
Results: On the 14th day after induction the findings demonstrated a significant increase in adipocyte-specific (PPARγ and LPL) gene expression compared to the control group (p<0.05) and a slight but not statistically significant decrease in the expression of the osteocyte-specific genes (RUNX2 and osteocalcin) (p>0.05).
Conclusion: The results suggest that B19V infection can promote the differentiation of hBM-MSCs towards adipocytes and affect the bone marrow microenvironment as well as hematopoiesis
{"title":"Parvovirus B19 Infection May Potentially Determine the Fate of Hematopoiesis by Altering the Human Bone Marrow Mesenchymal Stem Cells Differentiation","authors":"Azin Elmi, Amir Atashi, Nematollah Gheibi, Shahin Amiri, Monireh Ajami, Mansoureh Ajami, Razieh Mohammadihaji, Naeimeh Khodabandeloo, Mehdi Azad","doi":"10.18502/ijpho.v13i4.13770","DOIUrl":"https://doi.org/10.18502/ijpho.v13i4.13770","url":null,"abstract":"Background: Human bone marrow mesenchymal stem cells (hBM-MSCs), as supporters for hematopoiesis, differentiate into osteoblasts and adipocytes. Studies showed that infection of hBM-MSCs by Parvovirus B19 (B19V) can affect the differentiation capability of hBM-MSCs. This study aims to evaluate B19V effects on the differentiation of hBM-MSCs.
 Materials and Methods: In this experimental study hBM-MSCs were cultured up to passage 3. Nucleofection was subsequently employed to deliver a plasmid containing the B19V genome into the cells. The transfected cells were then differentiated into osteoblast and adipocyte lineages. qRT-PCR was then performed to analyze the differentiation 14 days after transfection.
 Results: On the 14th day after induction the findings demonstrated a significant increase in adipocyte-specific (PPARγ and LPL) gene expression compared to the control group (p<0.05) and a slight but not statistically significant decrease in the expression of the osteocyte-specific genes (RUNX2 and osteocalcin) (p>0.05).
 Conclusion: The results suggest that B19V infection can promote the differentiation of hBM-MSCs towards adipocytes and affect the bone marrow microenvironment as well as hematopoiesis","PeriodicalId":44212,"journal":{"name":"Iranian Journal of Pediatric Hematology and Oncology","volume":"52 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135198247","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Β-thalassemia is the severe form of genetic illnesses which decreases the hemoglobin synthesis. One of the major complications in thalassemic syndromes, including β-thalassemia major and intermedia is thromboembolic events. In addition, thromboembolic events are more common in non-transfusion-dependent thalassemia than those in well-transfusion-dependent β-thalassemia. A combination of hypercoagulable states including, abnormalities in red blood cells, and platelet, antithrombin III, protein C, and protein S, and splenectomy are involved in thrombotic events, but thromboembolic events can be prevented and treated in these patients via blood transfusion, hydroxyurea, anticoagulants, and aspirin. Moreover, recent studies have demonstrated the involvement of the brain lesion in β-thalassemia patients. The involvement of vascular events of brain in patients with β-thalassemia intermedia is 29-83%, but the rate of asymptomatic brain lesions in the healthy people is 0-11%. In addition, neurological complications which have been attributed to various factors are chronic hypoxia, iron overload, bone marrow expansion, and desferrioxamine neurotoxicity. This review evaluated thromboembolic events and neurological lesions in patients with β-thalassemia and its probable curative therapy.
{"title":"A review on thromboembolic events and neurological lesions in patients with β-thalassemia","authors":"Negin Kheiri, Morteza Zangeneh Soroush, Marzieh Aboutorabi","doi":"10.18502/ijpho.v13i4.13773","DOIUrl":"https://doi.org/10.18502/ijpho.v13i4.13773","url":null,"abstract":"Β-thalassemia is the severe form of genetic illnesses which decreases the hemoglobin synthesis. One of the major complications in thalassemic syndromes, including β-thalassemia major and intermedia is thromboembolic events. In addition, thromboembolic events are more common in non-transfusion-dependent thalassemia than those in well-transfusion-dependent β-thalassemia. A combination of hypercoagulable states including, abnormalities in red blood cells, and platelet, antithrombin III, protein C, and protein S, and splenectomy are involved in thrombotic events, but thromboembolic events can be prevented and treated in these patients via blood transfusion, hydroxyurea, anticoagulants, and aspirin. Moreover, recent studies have demonstrated the involvement of the brain lesion in β-thalassemia patients. The involvement of vascular events of brain in patients with β-thalassemia intermedia is 29-83%, but the rate of asymptomatic brain lesions in the healthy people is 0-11%. In addition, neurological complications which have been attributed to various factors are chronic hypoxia, iron overload, bone marrow expansion, and desferrioxamine neurotoxicity. This review evaluated thromboembolic events and neurological lesions in patients with β-thalassemia and its probable curative therapy.","PeriodicalId":44212,"journal":{"name":"Iranian Journal of Pediatric Hematology and Oncology","volume":"3 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135199967","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-01-15DOI: 10.18502/ijpho.v12i1.8364
M. Eini, M. Shoae, E. Miri-Moghaddam
Beta-thalassemia (β-thal) is a congenital hemoglobinopathy explained by a decreased level (β+) or absence (βο) of β-globin gene expression. Microcytic hypochromic anemia and various clinical symptoms comprising severe anemia to clinically nonsymptomatic features. Treatment with an ordered blood transfusion and iron chelator agents can decrease transfusion iron overload that causes normal maturation. These patients also are at high risk for secondary iron overload because of erythropheron (GF15–TWSG1) release from erythroblasts resulting in erythroid hyperplasia. Based on the previous studies, chemicals such as hydroxyurea and 5-azacytidine are useful in treating β-hemoglobinopathy, including β-thal and sickle cell disease (SCD). Regarding both side effects and lifelong treatment of these chemical components, researchers have recently regarded gene-based treatments. These techniques, such as micro RNA gene silencing, viral-mediated gene editing, and clustered regulatory interspaced short palindromic repeats (CRISPR)-CAS9 systems, are the most commonly used gene therapy methods. Nowadays, ɣ-globin (fetal globin) gene reactivation is one of the most popular treatments for β-thal. Researches showed that these gene modification methods for γ-globin gene reactivation are also useful in increasing hemoglobin F (HbF) and helping patients with β-thal. In this review study, new therapeutic approaches to manage this disorder are regarded.
{"title":"Therapeutic approaches in patients with β-thalassemia","authors":"M. Eini, M. Shoae, E. Miri-Moghaddam","doi":"10.18502/ijpho.v12i1.8364","DOIUrl":"https://doi.org/10.18502/ijpho.v12i1.8364","url":null,"abstract":"Beta-thalassemia (β-thal) is a congenital hemoglobinopathy explained by a decreased level (β+) or absence (βο) of β-globin gene expression. Microcytic hypochromic anemia and various clinical symptoms comprising severe anemia to clinically nonsymptomatic features. Treatment with an ordered blood transfusion and iron chelator agents can decrease transfusion iron overload that causes normal maturation. These patients also are at high risk for secondary iron overload because of erythropheron (GF15–TWSG1) release from erythroblasts resulting in erythroid hyperplasia. Based on the previous studies, chemicals such as hydroxyurea and 5-azacytidine are useful in treating β-hemoglobinopathy, including β-thal and sickle cell disease (SCD). Regarding both side effects and lifelong treatment of these chemical components, researchers have recently regarded gene-based treatments. These techniques, such as micro RNA gene silencing, viral-mediated gene editing, and clustered regulatory interspaced short palindromic repeats (CRISPR)-CAS9 systems, are the most commonly used gene therapy methods. Nowadays, ɣ-globin (fetal globin) gene reactivation is one of the most popular treatments for β-thal. Researches showed that these gene modification methods for γ-globin gene reactivation are also useful in increasing hemoglobin F (HbF) and helping patients with β-thal. In this review study, new therapeutic approaches to manage this disorder are regarded.","PeriodicalId":44212,"journal":{"name":"Iranian Journal of Pediatric Hematology and Oncology","volume":"64 1","pages":""},"PeriodicalIF":0.3,"publicationDate":"2022-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77929676","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-01-15DOI: 10.18502/ijpho.v12i1.8365
N. Reisi, Pardis Nematolahy
The development of secondary malignancy (SM) is the most worrisome long-term complication of childhood cancer. Acute myeloid leukemia is the most prevalent neoplasm that occurs after treatment with alkylating agents and topoisomerase II inhibitors. Pleuropulmonary blastoma (PPB) is a rare lung neoplasm in children. Type II and type III of this cancer are markedly aggressive and have a recurrent nature. Chemotherapy, radiation therapy, and hematopoietic stem cell transplant (HSCT) are treatment modalities that make these patients prone to secondary malignancy. Here was presented and discussed a case of myeloid leukemia 3.5 years after treatment of Pleuropulmonary blastoma in a 5.5-year-old boy who was a candidate for high dose chemotherapy and autologous stem cells transplant (auto-SCT) because of frequent recurrence and lack of response to chemotherapy and radiation therapy. It seems this is the first reported case of therapy-related myeloid leukemia (t-AML) after PPB in children. Awareness of the creation of this complication following administration of cytotoxic therapies in the treatment of solid tumors will increase physician attention in the selection of treatment modality as well as the counseling of patients at the time of diagnosis.
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Background: Osteoporosis is one of the late complications of β-Thalassemia major. The pathogenesis of osteoporosis depends on different factors. Ineffectiveness of hematopoiesis is the major factor, and the other factors are defected by hormonal functions or biochemical parameters. Osteoclasts hyperactivity in thalassemia increases the serum receptor activator of nuclear factor Kappa B ligand (RANKL), which plays a crucial role in bone development. This study aimed to evaluate the biochemical and hormonal parameters in patients with β-thalassemia major and their association with osteoporosis. Materials and Methods: In this case-control study, 52 patients with β-thalassemia major and 23 with thalassemia minor as controls were enrolled. The patients’ Bone Mineral Density (BMD) was measured using the Dual Energy X-ray absorptiometry (DEXA) method, and 6 mL peripheral blood of the patients and controls was obtained to detect hormonal and biochemical parameters. Data were analyzed using ANOVA, Spearman correlation coefficient, and T-test. Results: The mean of BMD in patients was 0.59±0.01 and 0.69±0.11 in femur and vertebrae, respectively. The biochemical parameters in the (patients/ controls) including calcium and alkaline phosphatase (ALK) were 9.1/ 10.2 mg/dL and 171.1/310 IU, respectively indicating a significant decrease (P< 0.05) compared to the controls. On the contrary, the mean levels of Ferritin and Zinc were 1914.18 µg/L and 113.92 mg/mL, respectively which were significantly increased (P= 0.015 and P=0.045, respectively). There was a negative correlation between the femurs BMD of patients with the RANKL level (r= - 0.8, p = 0.034) and the vertebrae BMD of patients with a Parathormone (PTH) level (r= - 0.8, P = 0.028). Conclusion: The study results indicated that the hyperactivity of RANKL and PTH in thalassemia patients might cause osteoporosis; therefore, detecting biomarkers mentioned above could be useful to diagnose osteoporosis.
背景:骨质疏松是重度β-地中海贫血的晚期并发症之一。骨质疏松症的发病机制取决于不同的因素。造血功能无效是主要因素，其他因素受激素功能或生化参数的影响。地中海贫血的破骨细胞过度活跃增加了核因子κ B配体(RANKL)的血清受体激活因子，这在骨发育中起着至关重要的作用。本研究旨在评价β-地中海贫血患者的生化和激素指标及其与骨质疏松症的关系。材料与方法:本病例对照研究纳入52例重度β-地中海贫血患者和23例轻度地中海贫血患者作为对照。采用双能x线骨密度仪(DEXA)测定患者骨密度(BMD)，取患者及对照组外周血6 mL检测激素及生化指标。数据分析采用方差分析、Spearman相关系数和t检验。结果:患者股骨骨密度平均值为0.59±0.01，椎骨骨密度平均值为0.69±0.11。(患者/对照组)钙和碱性磷酸酶(ALK)生化指标分别为9.1/ 10.2 mg/dL和171.1/310 IU，与对照组相比显著降低(P< 0.05)。相反，铁蛋白和锌的平均含量分别为1914.18µg/L和113.92 mg/mL，显著升高(P= 0.015和P=0.045)。RANKL水平患者股骨骨密度(r= - 0.8, p = 0.034)与甲状旁激素(PTH)水平患者椎骨骨密度(r= - 0.8, p = 0.028)呈负相关。结论:地中海贫血患者RANKL和PTH的高活性可能导致骨质疏松;因此，检测上述生物标志物可能有助于骨质疏松症的诊断。
{"title":"Evaluation of relationship between biochemical parameters and osteoporosis in patients with β-thalassemia major","authors":"M. Hamidpour, Fatemeh Jafari, Mahdieh Mehrpouri, Azita Azarkyvan, Davod Bashash, Aliakbar Khadem Maboudi","doi":"10.18502/ijpho.v12i1.8360","DOIUrl":"https://doi.org/10.18502/ijpho.v12i1.8360","url":null,"abstract":"Background: Osteoporosis is one of the late complications of β-Thalassemia major. The pathogenesis of osteoporosis depends on different factors. Ineffectiveness of hematopoiesis is the major factor, and the other factors are defected by hormonal functions or biochemical parameters. Osteoclasts hyperactivity in thalassemia increases the serum receptor activator of nuclear factor Kappa B ligand (RANKL), which plays a crucial role in bone development. This study aimed to evaluate the biochemical and hormonal parameters in patients with β-thalassemia major and their association with osteoporosis. \u0000Materials and Methods: In this case-control study, 52 patients with β-thalassemia major and 23 with thalassemia minor as controls were enrolled. The patients’ Bone Mineral Density (BMD) was measured using the Dual Energy X-ray absorptiometry (DEXA) method, and 6 mL peripheral blood of the patients and controls was obtained to detect hormonal and biochemical parameters. Data were analyzed using ANOVA, Spearman correlation coefficient, and T-test. \u0000Results: The mean of BMD in patients was 0.59±0.01 and 0.69±0.11 in femur and vertebrae, respectively. The biochemical parameters in the (patients/ controls) including calcium and alkaline phosphatase (ALK) were 9.1/ 10.2 mg/dL and 171.1/310 IU, respectively indicating a significant decrease (P< 0.05) compared to the controls. On the contrary, the mean levels of Ferritin and Zinc were 1914.18 µg/L and 113.92 mg/mL, respectively which were significantly increased (P= 0.015 and P=0.045, respectively). There was a negative correlation between the femurs BMD of patients with the RANKL level (r= - 0.8, p = 0.034) and the vertebrae BMD of patients with a Parathormone (PTH) level (r= - 0.8, P = 0.028). \u0000Conclusion: The study results indicated that the hyperactivity of RANKL and PTH in thalassemia patients might cause osteoporosis; therefore, detecting biomarkers mentioned above could be useful to diagnose osteoporosis.","PeriodicalId":44212,"journal":{"name":"Iranian Journal of Pediatric Hematology and Oncology","volume":"24 1","pages":""},"PeriodicalIF":0.3,"publicationDate":"2022-01-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81904638","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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