Sohier Yahia, Waleed Eldars, Heba Eldegla, Ahmed K Mansour, Mouna Guaida, Mohamed S A Abdelkader, Yahya Wahba
{"title":"Cell Death Markers in Children with Immune Thrombocytopenic Purpura: A Preliminary Study.","authors":"Sohier Yahia, Waleed Eldars, Heba Eldegla, Ahmed K Mansour, Mouna Guaida, Mohamed S A Abdelkader, Yahya Wahba","doi":"10.1007/s12288-023-01639-0","DOIUrl":"https://doi.org/10.1007/s12288-023-01639-0","url":null,"abstract":"<p><p>Immune thrombocytopenic purpura (ITP) is an autoimmune disease with possible dysregulation of the apoptotic pathways. We aimed to evaluate the possible role of some apoptotic markers (caspase 3, caspase 8 and BCL2) in the pathogenesis and course of ITP. We investigated some apoptotic markers (caspase 3, caspase 8 and BCL2) using the flow cytometry in 60 children with newly diagnosed ITP, 20 children with chemotherapy-related thrombocytopenia (CRT) and 20 healthy children. We also assessed the effects of intravenous immunoglobulin (IVIG) and methyl prednisolone therapies on the platelet apoptosis in children with newly diagnosed ITP. We demonstrated significantly higher values of caspase 3 in the newly diagnosed ITP group than control and CRT groups, and non-significantly higher values of caspase 8 in the ITP group than the healthy group. After IVIG treatment, the platelet count increased in all patients, and there was a significant decrease in caspase 3 and caspase 8 levels while BCL2 level increased. Regarding methylprednisolone treatment, there was a significant decrease in BCL2 and caspase 8 levels while caspase 3 levels did not significantly decrease. There is a possible role of the caspase dependent cell death pathway of the platelets in the occurrence of newly diagnosed ITP. There is heterogeneity in the apoptotic changes of newly diagnosed ITP children who received IVIG versus those who received methylprednisolone.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542074/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41162254","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Samarjit Maharana, Chitrali Laha Roy, Kamal Kishor, Ravi Ranjan, Firdos Ahmad, Manoranjan Mahapatra, Renu Saxena, Meganathan Kannan
{"title":"Depolarized Mitochondrial Membrane Potential and Elevated Calcium in Platelets of Sickle Cell Disease.","authors":"Samarjit Maharana, Chitrali Laha Roy, Kamal Kishor, Ravi Ranjan, Firdos Ahmad, Manoranjan Mahapatra, Renu Saxena, Meganathan Kannan","doi":"10.1007/s12288-023-01640-7","DOIUrl":"https://doi.org/10.1007/s12288-023-01640-7","url":null,"abstract":"<p><p>Hemolysis, a crucial feature of Sickle cell disease (SCD), is a key player for cellular activation leading to various complications including thrombosis. In response to hemolysis, platelets get activated and release components that are necessary for further platelet activation and aggregation. Thus, it is believed that platelets contribute to the development of thrombotic complications. Platelets in SCD are expected to be affected due to common cause of hemolysis. To measure the surface markers of platelets including P-Selectin, Phosphatidyl Serine and integrin αIIbβ3 in SCD patients and healthy controls in order to understand the status of the platelets in SCD. To measure the surface markers of activated platelets using flow cytometry. Since mitochondria and calcium play an important role in cellular functions, the mitochondrial membrane potential and calcium content of platelets in SCD were also evaluated using flow cytometry. In the present study, we have observed significant increase of calcium level in SCD platelets. Further, the loss of mitochondrial membrane potential in SCD platelets was found to be significantly higher when compared to platelets of healthy controls. Though the surface markers of activated platelets in SCD remain unchanged, increased level of calcium and mitochondrial membrane potential loss suggest that the platelets in SCD are more prone to become activated. In order to understand the status of the platelets in SCD, apart from the surface markers, it is also important to assess the calcium levels and mitochondrial membrane potential of platelets.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542052/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41155733","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Analysis of an Inherited FXII Deficiency Pedigree Associated with Double Heterozygous Mutations in the F12 Gene.","authors":"Manlin Zeng, Kaiqi Jia, Haixiao Xie, Yaosheng Xie, Lihong Yang, Yuan Chen, Yanhui Jin, Mingshan Wang","doi":"10.1007/s12288-023-01666-x","DOIUrl":"https://doi.org/10.1007/s12288-023-01666-x","url":null,"abstract":"<p><p>This article is intended to identify the potential mutations of the FXII gene (<i>F12</i>) in an inherited FXII deficiency pedigree and illuminate the pathogenesis of the disease. The coagulation FXII activity (FXII:C) and FXII antigen (FXII:Ag) were inspected by one-stage clotting assay and enzyme-linked immunosorbent assay(ELISA), respectively. Polymerase chain reaction amplification (PCR) was performed and the <i>F12</i> gene was sequenced directly. A molecular model of FXIIprotein was established for further analysis. ClustalX-2.1-win and online bioinformatic software were used to estimate the conservatism and possible impact of the protein change. The proband presented prolonged APTT(180 s) and extreme low FXII:C and FXII:Ag (both < 1%, reference range:72-113%). A compound heterozygous were found by the direct sequencing of the <i>F12</i> gene. One was a deletion mutation c.1792_1796delGTCTA, which is a novel mutation; the other was an insertion mutation, c.1092_1093insC. Bioinformatic and modeling analyses indicated that the the two frameshift mutations may be deleterious and possibly alter the structure and the function of the protein. The mutations c.1792_1796delGTCTA and c.1092_1093insC could be the main causes of reducing FXII in this pedigree, and c.1792_1796delGTCTA mutation was the first report in the world.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542433/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41167396","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Salih Güler, Aytül Temuroğlu, Melike Sezgin Evim, Adalet Meral Günes
{"title":"Incidence and Management of Thromboembolism in Patients with Acute Leukemia.","authors":"Salih Güler, Aytül Temuroğlu, Melike Sezgin Evim, Adalet Meral Günes","doi":"10.1007/s12288-023-01642-5","DOIUrl":"https://doi.org/10.1007/s12288-023-01642-5","url":null,"abstract":"<p><p>Thromboembolic events (TE) in childhood are relatively rare but, serious complications of acute leukemia. The aim was to define the incidence and risk factors of thrombosis in children with leukemias. The electronic files of pediatric denovo/relapsed acute leukemia patients aged below 18 years, treated between 2011 and 2021 were retrospectively evaluated for thrombotic attacks. Thirty out of 469 patients developed 35 thrombotic events. The median age at the time of the TE was 11.8 (2-17.6) years, and the median time from diagnosis to TE was 9 (0-58) months. The frequency of TE was found at 7.4% (n = 35/469). When catheter related (n = 13) events, superficial venous events (n = 10), and arterial central nervous system thrombosis (n = 1) were excluded, the frequency of TE was decreased to 2.3% (n = 11/469). Children older than 10 years old (13.8%; n = 21/152) had significantly higher thromboembolic events than the others (4.4%; n = 14/317) (p = 0.03). The majority of attacks were symptomatic 66% (n = 23/35). The most common complaints were local pain, swelling, and redness 52% (n = 12/23). The majority of attacks in patients with relapsed (75%; 6/8) and newly diagnosed acute lymphoblastic leukemia (40%; 10/25%) developed during the induction phase. Thrombosis recurred in 13.3% (n = 4/30) of cases more than once. Thrombotic attacks were successfully treated with low molecular weight heparin 60% (n = 21/35), and recombinant tissue plasminogen activator 17% (n = 6/35). None of the children were lost due to thrombosis. Thrombosis is an important complication during acute leukemia treatment. Successful results are obtained with early diagnosis and treatment attempts by creating awareness.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542053/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41116961","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mahnaz Ghaljeh, Fatemeh Kord Salarzehi, Sajad Salehipour
{"title":"The Effect of Benson Relaxation Technique on Anxiety and Quality of Life in Patients with Thalassemia Major: A Clinical Trial.","authors":"Mahnaz Ghaljeh, Fatemeh Kord Salarzehi, Sajad Salehipour","doi":"10.1007/s12288-023-01633-6","DOIUrl":"https://doi.org/10.1007/s12288-023-01633-6","url":null,"abstract":"<p><p>Thalassemia major is the most common chronic blood disease in the world, especially in Asia and Iran, and it gives rise to anxiety and reduces quality of life [QOL] in patients. The purpose of this study was to determine the effect of Benson relaxation technique on anxiety and QOL in patients with thalassemia major. This semi-experimental clinical trial study was conducted on 140 patients with thalassemia major in two intervention groups [n = 70] and control group [n = 70] in Ali Asghar Hospital, Zahedan.The data were collected using a demographic information form, the Spielberger State-Trait Anxiety Inventory [STAI], and the World Health Organization Quality of Life-BREF [WHOQOL-BREF] and self-report checklist. The control group received routine care, whereas the intervention group, we first completed the questionnaires; then, Benson relaxation technique was taught to each patient in three one-hour sessions in the presence of a family member for three consecutive days. Finally, they were asked to practice this technique twice a day for 12 weeks. The questionnaires were completed again one and three months after the intervention. Data analysis showed no significant difference between the mean scores of anxiety and QOL and its dimensions in the two groups at baseline [<i>P</i> > 0.001]. One and three months after the relaxation technique, however, the intervention group experienced a statistically significant difference in the mean scores of anxiety and QOL and its dimensions [<i>P</i> < 0.001]. The results confirmed that Benson Relaxation Technique reduces anxiety and improves the QOL of patients with thalassemia major. <i>Clinical Trials Registration</i>: IRCT20200926048842N2.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542056/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41144589","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Evaluation of Diagnostic Usefulness of CD200 Expression in B-cell Chronic Lymphoproliferative Disorders.","authors":"Abhishek Purohit, Manali Satiza, Venkatesan Somasundaram, Rahul Sharma, Pravas Mishra, Tulika Seth, Seema Tyagi, Manoranjan Mahapatra, Hara Prasad Pati, Renu Saxena","doi":"10.1007/s12288-022-01622-1","DOIUrl":"https://doi.org/10.1007/s12288-022-01622-1","url":null,"abstract":"<p><p>Immunophenotyping by flow cytometry (FCM) is a useful diagnostic tool for the evaluation of mature B-cell neoplasms (MBN). Here, CD200 expression may play a significant role and improve the distinction between various MBNs, but any potential as a prognostic marker is yet to be established. The present prospective study was conducted on all the suspected cases of MBNs. Immunophenotyping was done using a BD FACS Canto FCM using a panel of 4 to 6 color combinations of monoclonal antibodies; CD45, CD34, CD5, CD19, CD20, CD22, CD23, CD79b, FMC7, CD10, CD38, ZAP70, CD200, IgG, IgM, CD25, CD103, CD2, CD3, CD11c as well as κ and λ light chains. CD200 expression was compared in different subgroups. Of the total of 130 cases included in the study, CD200 was positive in 118 cases (90%). CD200 was expressed in 100% of the cases of CLL(86 cases), atypical CLL(06 cases), HCL(14 cases), FL(02 cases), SMZL(04 cases), LPL (01 case), and low-grade NHL (05 cases), with the highest intensity of fluorescence in HCL followed by CLL. All the cases of MCL and PLL were exclusively negative for CD200. In conclusion, the results of the present study support inclusion of this marker in the flow cytometric panels for the differential diagnosis of MBNs.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542070/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41150223","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Inhibition of PI3K Signaling Intensified the Antileukemic Effects of Pioglitazone: New Insight into the Application of PPARγ Stimulators in Acute Lymphoblastic Leukemia.","authors":"Yazdan Mokhtari, Amir-Mohammad Yousefi, Davood Bashash","doi":"10.1007/s12288-023-01650-5","DOIUrl":"https://doi.org/10.1007/s12288-023-01650-5","url":null,"abstract":"<p><p>Over the past two decades, molecular targeted therapy has revolutionized the landscape of cancer treatment due to lower side effects as well as higher anticancer effects. Peroxisome proliferator-activated receptor gamma (PPARγ) is a member of the nuclear hormone receptor which plays a crucial role in cell proliferation and death and the efficacy of PPARγ ligands either as monotherapy or in combination with traditional chemotherapy drugs has been proved by recent studies. In this study, we aimed to investigate the effects of pioglitazone, a well-known PPARγ stimulator, in ALL-derived NALM6 cells by using trypan blue assay, MTT assay, and flow cytometry analysis. Moreover, to investigate the molecular mechanism action of pioglitazone in these cells, we assessed the possible alterations in the expression of some target genes which regulate cell proliferation, apoptosis, and autophagy system. Our result demonstrated that pioglitazone induced a remarkable antileukemic effect on NALM6 cells through a PTEN-mediated manner. Based on the fact that PI3K hyperactivation is one of the main properties of ALL cells, the effects of PI3K inhibition using CAL-101 on pioglitazone-induced cytotoxicity were evaluated by combinatorial experiments. Moreover, the result of cell cycle assay and qRT-PCR demonstrated that pioglitazone-CAL-101 induced antileukemic effect mainly through induction of p21 and p27-mediated G1 arrest. Additionally, our result showed that inhibition of proteasome and autophagy system, two main cellular processes, increased the antileukemic effects of the agents. Taken together, we suggest a novel therapeutic application for PPARγ stimulators as a single agent or in combination with PI3K inhibitors that should be clinically evaluated in ALL patients.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542079/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41169313","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ivan Krečak, Hrvoje Holik, Martina Morić Perić, Ivan Zekanović, Božena Coha, Marko Lucijanić
{"title":"Chronic Kidney Disease has a Higher Prevalence in Polycythemia Vera than in Secondary Polycythemia, a Matched Case-Control Analysis.","authors":"Ivan Krečak, Hrvoje Holik, Martina Morić Perić, Ivan Zekanović, Božena Coha, Marko Lucijanić","doi":"10.1007/s12288-022-01624-z","DOIUrl":"https://doi.org/10.1007/s12288-022-01624-z","url":null,"abstract":"","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542037/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41157304","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Investigating the Correlation Between HLA-II Gene Polymorphism and RhE Alloimmunization in Pregnant Chinese Women.","authors":"Wenling Shang, Guojin Ou, Xin Ji, Jian Chen, Jue Wang, Yongmei Jiang","doi":"10.1007/s12288-023-01632-7","DOIUrl":"https://doi.org/10.1007/s12288-023-01632-7","url":null,"abstract":"<p><p>The Rhesus (Rh) blood group is a significant and complicated biological system in humans. Incompatible transfusion or pregnancy with Rh antigens can lead to the production of alloantibodies, among which the anti-E antibody is prevalent. The relationship between Anti-E antibody and HLA-II gene polymorphism in Chinese pregnant women is worth exploring. Our aim in this study was to verify the correlation between HLA-II gene polymorphisms and RhE alloimmunization in pregnant Chinese women through HLA-II typing and DR-RhE structural prediction. In total, 94 anti-E-negative pregnant women and 103 anti-E-positive pregnant women were enrolled from Southwest China Second Hospital, and HLA-II genotyping was performed using next-generation sequencing. NetMHCpan software was used to predict the binding of E -derived anchoring peptides to HLA-DRB1 molecules. AlphaFold was used to analyze the differences in antigen presentation based on the structure of major histocompatibility complex peptides. The HLA-DRB1*09:01-DQA1*03:02-DQB1*03:03 haplotype showed a significant positive association with anti-E. One E-derived anchoring peptide (219FWPSVNSPL227) was predicted to bind to the HLA-DRB1*09:01 molecule. The interaction between the 60Ser of DR9 and 226pro of RhE comprised one hydrogen bond. This study demonstrated that HLA-II haplotypes are associated with allo-anti-E antibodies in pregnant women from Sichuan Province, China. The HLA-DRB1*09:01-DQA1*03:02-DQB1*03:03 phenotype may enhance the formation of anti-E alloantibodies, and the HLA-DRB1*09:01 molecule may play a key role in alloimmunity.</p>","PeriodicalId":49188,"journal":{"name":"Indian Journal of Hematology and Blood Transfusion","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10542046/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41157305","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}