Mediterranean Journal of Hematology and Infectious Diseases最新文献

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A multicenter ICET-A study on age at menarche and menstrual cycles in patients with transfusion-dependent thalassemia (TDT) who started early chelation therapy with different chelating agents 一项多中心ICET-A研究:输血依赖性地中海贫血(TDT)患者早期开始不同螯合剂螯合治疗的月经初潮年龄和月经周期
4区 医学
Mediterranean Journal of Hematology and Infectious Diseases Pub Date : 2023-10-30 DOI: 10.4084/mjhid.2023.058
SALVATORE DI MAIO, VINCENZO DE SANCTIS, PIERLUIGI MARZUILLO, CHRISTOS KATTAMIS, SHAHINA DAAR, MEHERAN KARIMI, SAKI FOROUGH, ATANAS BANKEV, VALERIA KALEVA, SOTEROULA CHRISTOU, CARMELO FORTUGNO, POLYXENI DELAPORTA, ASHRAF T SOLIMAN, PLOUTARCHOS TZOULIS
{"title":"A multicenter ICET-A study on age at menarche and menstrual cycles in patients with transfusion-dependent thalassemia (TDT) who started early chelation therapy with different chelating agents","authors":"SALVATORE DI MAIO, VINCENZO DE SANCTIS, PIERLUIGI MARZUILLO, CHRISTOS KATTAMIS, SHAHINA DAAR, MEHERAN KARIMI, SAKI FOROUGH, ATANAS BANKEV, VALERIA KALEVA, SOTEROULA CHRISTOU, CARMELO FORTUGNO, POLYXENI DELAPORTA, ASHRAF T SOLIMAN, PLOUTARCHOS TZOULIS","doi":"10.4084/mjhid.2023.058","DOIUrl":"https://doi.org/10.4084/mjhid.2023.058","url":null,"abstract":"Abstract. Objective: To evaluate the age at menarche and menstrual characteristics in patients with transfusion-dependent thalassemia (TDT) who started early chelation therapy (≤ 3 years) with a variety of chelating agents. Design: A retrospective multicenter study promoted by International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A). Setting: Eight of 13 International Thalassemia Centers (61.5%) in the ICET-A Network participated. Patients: Fifty-seven female TDT patients, aged 11 to 26 years, were enrolled in the study. Seven patients were excluded, 4 who were still prepubertal (age 12-14 years) and 3 with primary amenorrhea. The remaining 50 patients were from Iran (33 patients), 9 from Bulgaria, 8 from Greece, 4 from Oman, 2 from Cyprus, and 1 from Italy. Results: At start of chelation therapy, 22 patients received desferrioxamine mesylate (DFO), 26 deferasirox (DFX) and 2 deferiprone (DFP). All fifty TDT patients developed spontaneous menarche at a mean age of 14.2 ± 2.24 years (range 9 – 20). A significant positive correlation was observed between age at menarche and serum ferritin (SF) levels (r: 0. 41, P: 0.005). Thirty-two patients (64%) reported regular menstrual cycles, 7 (14 %) oligomenorrhea, 3 (6%) short/light menses (hypomenorrhea), and 8 (16%) secondary amenorrhea (SA) (16%). Conclusions: Early chelation does not necessary correlate with efficient chelation during pubertal age. Delayed menarche, related to high SF levels, was still frequent in most Centers and was a forerunner of irregular menstrual cycles, SA and associated complications. Neglecting the importance of adherence to iron chelation therapy (ICT), despite innovative and expensive therapies, may lead to complications and decreased quality of life.
 
 Key words: Transfusion-dependent thalassemia, menarche, menstrual cycles, iron chelation therapy (ICT), iron overload, adherence to treatment.","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136102296","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
CAR-T CELL THERAPY IN LARGE B CELL LYMPHOMA Car-t细胞治疗大b细胞淋巴瘤
4区 医学
Mediterranean Journal of Hematology and Infectious Diseases Pub Date : 2023-10-30 DOI: 10.4084/mjhid.2023.066
Ugo Testa, Germana Castelli, Giuseppe Leone, Elvira Pelosi, Germana Castelli, Stefan Hohaus
{"title":"CAR-T CELL THERAPY IN LARGE B CELL LYMPHOMA","authors":"Ugo Testa, Germana Castelli, Giuseppe Leone, Elvira Pelosi, Germana Castelli, Stefan Hohaus","doi":"10.4084/mjhid.2023.066","DOIUrl":"https://doi.org/10.4084/mjhid.2023.066","url":null,"abstract":"Large B-cell lymphomas (LBCLs) are among the most frequent (about 30%) non-Hodgkin’s lymphoma. Despite the aggressive behavior of these lymphomas, more than 60% of patients can be cured with first-line chemoimmunotherapy using the R-CHOP regimen. Patients with refractory or relapsing disease show a poor outcome even when treated with second-line therapies.
 CD19-targeted chimeric antigen receptor (CAR) T-cells are emerging as an efficacious second-line treatment strategy for patients with LBCL. Three CD19-CAR-T-cell products received FDA and EMA approval. The use of CAR-T cell therapy has also been explored for the treatment of high-risk LBCL patients in the first-line setting and for patients with central nervous system involvement.
 Although CD19-CAR-T therapy has transformed the care of refractory/relapsed LBCL, about 60% of these patients will ultimately progress or relapse following CD19-CAR-T: therefore, it is fundamental to identify predictive criteria of response to CAR-T therapy and to develop salvage therapies for patients relapsing after CD19-CAR-T therapies. Moreover, ongoing clinical trials are evaluating bispecific CAR-T cells targeting both CD19 and CD20 or CD19 and CD22 as a tool to improve the therapeutic efficacy and to reduce the number of refractory/relapsing patients.","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136022973","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
NOVEL MUTATIONS IN THE NON-STRUCTURE PROTEIN 2 OF SARS-CoV-2 SARS-CoV-2非结构蛋白2的新突变
4区 医学
Mediterranean Journal of Hematology and Infectious Diseases Pub Date : 2023-10-16 DOI: 10.4084/mjhid.2023.059
Mohsen Nakhaei, Zohreh-Al-Sadat Ghoreshi, Mohammad Rezaei Zadeh Rukerd, Hedyeh Askarpour, None Nasir
{"title":"NOVEL MUTATIONS IN THE NON-STRUCTURE PROTEIN 2 OF SARS-CoV-2","authors":"Mohsen Nakhaei, Zohreh-Al-Sadat Ghoreshi, Mohammad Rezaei Zadeh Rukerd, Hedyeh Askarpour, None Nasir","doi":"10.4084/mjhid.2023.059","DOIUrl":"https://doi.org/10.4084/mjhid.2023.059","url":null,"abstract":"Background: Mutation in the genome of SARS-CoV-2 may play a role in immune evasion, pathogenicity and speed of its transmission. Our investigation aimed to evaluate the mutations that exist in the nsp2.
 Materials and Method: RNA was extracted from nasopharyngeal swabs from 100 COVID-19 patients. RT-PCR was performed on all samples using nsp2 specific primers. Following gel electrophoresis, the bands were cut, purified, and sequenced using Sanger method. After sequencing, 90 sequences could be used for further analysis. Bioinformatics analysis was conducted to investigate the effect of mutations on protein structure, prediction of homology models, phylogeny tree.
 Results: The patients' mean age wa
 Background: Mutation in the genome of SARS-CoV-2 may play a role in immune evasion, pathogenicity and speed of its transmission. Our investigation aimed to evaluate the mutations that exist in the nsp2.
 Materials and Method: RNA was extracted from nasopharyngeal swabs from 100 COVID-19 patients. RT-PCR was performed on all samples using nsp2 specific primers. Following gel electrophoresis, the bands were cut, purified, and sequenced using Sanger method. After sequencing, 90 sequences could be used for further analysis. Bioinformatics analysis was conducted to investigate the effect of mutations on protein structure, prediction of homology models, phylogeny tree.
 Results: The patients' mean age was 51.08. The results revealed that 8 of the 17 nsp2 mutations (R207C, T224I, G262V, T265I, K337D, N348S, G392D, and I431M) were missense. One deletion was also found in nsp2. Among nsp2 missense mutations studied, K337D and G392D increased structural stability while the others decreased it. The homology-designed models demonstrated that the homologies were comparable to the sequences of the Wuhan-HU-1 virus.
 Conclusion: Our study suggested that the mutations as K337D and G392D modulate the stability of nsp2 and tracking viral evolution should be implemented and vaccine development updated.
 s 51.08. The results revealed that 8 of the 17 nsp2 mutations (R207C, T224I, G262V, T265I, K337D, N348S, G392D, and I431M) were missense. One deletion was also found in nsp2. Among nsp2 missense mutations studied, K337D and G392D increased structural stability while the others decreased it. The homology-designed models demonstrated that the homologies were comparable to the sequences of the Wuhan-HU-1 virus.
 Conclusion: Our study suggested that the mutations as K337D and G392D modulate the stability of nsp2 and tracking viral evolution should be implemented and vaccine development updated.","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136183736","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
TP53-Mutated Myelodysplasia and Acute Myeloid Leukemia. TP53突变骨髓增生异常和急性髓性白血病
IF 2 4区 医学
Mediterranean Journal of Hematology and Infectious Diseases Pub Date : 2023-07-01 eCollection Date: 2023-01-01 DOI: 10.4084/MJHID.2023.038
Ugo Testa, Germana Castelli, Elvira Pelosi
{"title":"TP53-Mutated Myelodysplasia and Acute Myeloid Leukemia.","authors":"Ugo Testa, Germana Castelli, Elvira Pelosi","doi":"10.4084/MJHID.2023.038","DOIUrl":"10.4084/MJHID.2023.038","url":null,"abstract":"<p><p>TP53-mutated myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) form a distinct and heterogeneous group of myeloid malignancies associated with poor outcomes. Studies carried out in the last years have in part elucidated the complex role played by TP53 mutations in the pathogenesis of these myeloid disorders and in the mechanisms of drug resistance. A consistent number of studies has shown that some molecular parameters, such as the presence of a single or multiple TP53 mutations, the presence of concomitant TP53 deletions, the association with co-occurring mutations, the clonal size of TP53 mutations, the involvement of a single (monoallelic) or of both TP53 alleles (biallelic) and the cytogenetic architecture of concomitant chromosome abnormalities are major determinants of outcomes of patients. The limited response of these patients to standard treatments, including induction chemotherapy, hypomethylating agents and venetoclax-based therapies and the discovery of an immune dysregulation have induced a shift to new emerging therapies, some of which being associated with promising efficacy. The main aim of these novel immune and nonimmune strategies consists in improving survival and in increasing the number of TP53-mutated MDS/AML patients in remission amenable to allogeneic stem cell transplantation.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/87/c9/mjhid-15-1-e2023038.PMC10332352.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10353069","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}
引用次数: 0
The Impact of Human Platelet Antigen Allele on Antiplatelet Antibodies and Cryoglobulins in Patients with Primary Immune Thrombocytopenia and Hepatitis C Virus-Associated Immune Thrombocytopenia. 人血小板抗原等位基因对原发性免疫性血小板减少症和丙型肝炎病毒相关免疫性血小板降低症患者的抗血小板抗体和冷球蛋白的影响。
IF 3.2 4区 医学
Mediterranean Journal of Hematology and Infectious Diseases Pub Date : 2023-05-01 eCollection Date: 2023-01-01 DOI: 10.4084/MJHID.2023.030
Cih-En Huang, Yi-Yang Chen, Jung-Jung Chang, Yu-Ying Wu, Wei-Ming Chen, Ying-Hsuan Wang, Min-Chi Chen, Chang-Hsien Lu, Chung-Sheng Shi, Chih-Cheng Chen
{"title":"The Impact of Human Platelet Antigen Allele on Antiplatelet Antibodies and Cryoglobulins in Patients with Primary Immune Thrombocytopenia and Hepatitis C Virus-Associated Immune Thrombocytopenia.","authors":"Cih-En Huang,&nbsp;Yi-Yang Chen,&nbsp;Jung-Jung Chang,&nbsp;Yu-Ying Wu,&nbsp;Wei-Ming Chen,&nbsp;Ying-Hsuan Wang,&nbsp;Min-Chi Chen,&nbsp;Chang-Hsien Lu,&nbsp;Chung-Sheng Shi,&nbsp;Chih-Cheng Chen","doi":"10.4084/MJHID.2023.030","DOIUrl":"10.4084/MJHID.2023.030","url":null,"abstract":"<p><strong>Background and objectives: </strong>Human platelet antigens (HPAs) are alloantigens associated with antiplatelet alloantibodies and the risk of immune thrombocytopenia (ITP). However, few studies have investigated associations among HPAs, antiplatelet autoantibodies, and cryoglobulins.</p><p><strong>Methods: </strong>We enrolled 43 patients with primary ITP, 47 with hepatitis C virus-associated ITP (HCV-ITP), 21 with hepatitis B virus-associated ITP (HBV-ITP), 25 controls with HCV, and 1013 normal controls. We analyzed HPA allele frequencies, including HPA1-6 and 15, antiplatelet antibodies binding to platelet glycoprotein (GP) IIb/IIIa, Ia/IIa, Ib/IX, IV, human leukocyte antigen class I, cryoglobulin IgG/A/M, and their associations with thrombocytopenia.</p><p><strong>Results: </strong>In the ITP cohort, HPA2ab, rather than HPA2aa, predicted a low platelet count. HPA2b was associated with the risk of developing ITP. HPA15b was correlated with multiple antiplatelet antibodies. In HCV-ITP patients, HPA3b was correlated with anti-GPIIb/IIIa antibodies. HCV-ITP patients with anti-GPIIb/IIIa antibodies had a higher positive rate of cryoglobulin IgG and IgA compared with those without anti-GPIIb/IIIa antibodies. Overlapping detection was also found among other antiplatelet antibodies and cryoglobulins. Like the antiplatelet antibodies, cryoglobulins were associated with clinical thrombocytopenia, implying their close relationship. Finally, we extracted cryoglobulins to confirm the exhibition of cryoglobulin-like antiplatelet antibodies. In contrast, in primary ITP patients, HPA3b was correlated with cryoglobulin IgG/A/M rather than anti-GPIIb/IIIa antibodies.</p><p><strong>Conclusion: </strong>HPA alleles were associated with antiplatelet autoantibodies and had different impacts in primary ITP and HCV-ITP patients. HCV-ITP was considered to be a symptom of mixed cryoglobulinemia in HCV patients. The pathophysiology may differ between these two groups.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/43/c5/mjhid-15-1-e2023030.PMC10171212.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9469377","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}
引用次数: 0
Phenotypic and Molecular Detection of Biofilm Formation in Methicillin-Resistant Staphylococcus Aureus Isolated from Different Clinical Sources in Erbil City. 埃尔比勒市不同临床来源耐甲氧西林金黄色葡萄球菌生物膜形成的表型和分子检测
IF 3.2 4区 医学
Mediterranean Journal of Hematology and Infectious Diseases Pub Date : 2023-01-01 DOI: 10.4084/MJHID.2023.016
Pishtiwan Ahmad Hamad
{"title":"Phenotypic and Molecular Detection of Biofilm Formation in Methicillin-Resistant <i>Staphylococcus Aureus</i> Isolated from Different Clinical Sources in Erbil City.","authors":"Pishtiwan Ahmad Hamad","doi":"10.4084/MJHID.2023.016","DOIUrl":"https://doi.org/10.4084/MJHID.2023.016","url":null,"abstract":"<p><strong>Background: </strong><i>Staphylococcus aureus</i> is an important causative pathogen. The production of biofilms is an important factor and makes these bacteria resistant to antimicrobial therapy.</p><p><strong>Objectives: </strong>the current study aimed to assess the prevalence of resistance to antibacterial agents and to evaluate the phenotypic and genotypic characterization of biofilm formation among <i>S. aureus</i> strains.</p><p><strong>Methods: </strong>This study included 50 isolates of Methicillin-resistant <i>S. aureus</i> (MRSA) and Methicillin-Susceptible <i>S. aureus</i> (MSSA). <i>S. aureus</i> was identified by molecular and conventional methods, and antimicrobial resistance was tested with a disc diffusion method. The biofilm formation was performed through the Microtiter plate method. Strains were subjected to PCR to determine the presence of <i>nuc</i>, <i>mecA</i>, <i>icaA</i>, <i>icaB</i>, <i>icaC</i>, and <i>icaD</i> genes.</p><p><strong>Results: </strong>Of the 50 <i>S. aureus</i> isolates, 32(64%) and 18(36%) were MRSA and MSSA, respectively. A large number of MRSA and MSSA isolates showed resistance to Penicillin and Azithromycin, and a lower number of MRSA and MSSA isolates showed resistance to Amikacin Gentamicin. None of the isolates was resistant to Vancomycin. The MRSA strains had significantly higher resistance against antibiotics than MSSA strains (P = 0.0154). All isolates (MRSA and MSSA) were able to produce biofilm with levels ranging from strong (31.25 %), (16.6%) to moderate (53.12%), (50%) to weak (15.6%), (33.3%) respectively. The MRSA strains had a significantly higher biofilm formation ability than the MSSA strains (P = 0.0079). The biofilm-encoding genes were detected among isolates with different frequencies. The majority of <i>S. aureus</i> isolates, 42 (84%), were positive for the icaA. The prevalence rates of the icaB, icaC and icaD genes were found to be 37 (74%), 40 (80%) and 41 (82%), respectively.</p><p><strong>Conclusions: </strong>The prevalence of biofilm encoding genes associated with multidrug resistance in <i>S. aureus</i> strains is high. Therefore, identifying epidemiology, molecular characteristics, and biofilm management of <i>S. aureus</i> infection would be helpful.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/51/c0/mjhid-15-1-e2023016.PMC10000948.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9095533","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}
引用次数: 1
Quantitative Analysis of Liver Iron Deposition Based on Dual-Energy CT in Thalassemia Patients. 基于双能CT的地中海贫血患者肝铁沉积定量分析。
IF 3.2 4区 医学
Mediterranean Journal of Hematology and Infectious Diseases Pub Date : 2023-01-01 DOI: 10.4084/MJHID.2023.020
Fengming Xu, Cheng Tang, Yiling Huang, Linlin Liang, Fuling Huang, Gaohui Yang, Peng Peng
{"title":"Quantitative Analysis of Liver Iron Deposition Based on Dual-Energy CT in Thalassemia Patients.","authors":"Fengming Xu,&nbsp;Cheng Tang,&nbsp;Yiling Huang,&nbsp;Linlin Liang,&nbsp;Fuling Huang,&nbsp;Gaohui Yang,&nbsp;Peng Peng","doi":"10.4084/MJHID.2023.020","DOIUrl":"https://doi.org/10.4084/MJHID.2023.020","url":null,"abstract":"<p><strong>Background: </strong>To explore the feasibility and accuracy of liver iron deposition based on dual-energy CT in thalassemia patients.</p><p><strong>Materials and methods: </strong>105 thalassemia patients were examined with dual-energy CT and MR liver scanning. Dual-energy CT was performed to measure CT values on 80kVp, 140kVp, and virtual iron content (VIC) imaging; ΔH was figured out by the difference in CT values between 80kVp and 140kVp. Using the liver iron concentration (LIC) obtained by FerriScan as a gold standard, the correlation between CT measurements and LIC was evaluated. Receiver operating characteristic (ROC) analysis was used to evaluate the diagnostic performance for dual-energy CT in liver iron quantification and stratification.</p><p><strong>Results: </strong>The correlation analysis between CT measurements and LIC showed that 80kVp, 140kVp, VIC, and ΔH all had a high positive correlation with LIC (<i>P</i><0.001). The correlation analysis among different degree groups of VIC, ΔH, and LIC showed that the normal, moderate, and severe groups of VIC and ΔH had moderate or high positive correlations with that of LIC (<i>P</i><0.01), but the mild group had no correlation (<i>P</i>>0.05). ROC analysis revealed that the corresponding optimal cutoff value of VIC was -2.8, 6.3,11.9 HU (corresponds to 3.2,7.0,15.0 mg/g dry weight) respectively, while the ΔH were 5.1, 8.4, 17.8HU, respectively. The area under the receiver operating characteristic curves (AUCs) for both VIC and ΔH increased with LIC thresholds.</p><p><strong>Conclusion: </strong>Dual-energy CT can accurately quantify and stratify liver iron deposition, contributing to predicting the status of liver iron deposition in thalassemia patients.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ad/5c/mjhid-15-1-e2023020.PMC10000822.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9095535","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}
引用次数: 0
Genetic Predisposition to Hematologic Malignancies in Childhood and Adolescence. 儿童和青少年血液恶性肿瘤的遗传易感性。
IF 3.2 4区 医学
Mediterranean Journal of Hematology and Infectious Diseases Pub Date : 2023-01-01 DOI: 10.4084/MJHID.2023.032
Francesco Fabozzi, Angela Mastronuzzi
{"title":"Genetic Predisposition to Hematologic Malignancies in Childhood and Adolescence.","authors":"Francesco Fabozzi,&nbsp;Angela Mastronuzzi","doi":"10.4084/MJHID.2023.032","DOIUrl":"https://doi.org/10.4084/MJHID.2023.032","url":null,"abstract":"<p><p>Advances in molecular biology and genetic testing have greatly improved our understanding of the genetic basis of hematologic malignancies and have enabled the identification of new cancer predisposition syndromes. Recognizing a germline mutation in a patient affected by a hematologic malignancy allows for a tailored treatment approach to minimize toxicities. It informs the donor selection, the timing, and the conditioning strategy for hematopoietic stem cell transplantation, as well as the comorbidities evaluation and surveillance strategies. This review provides an overview of germline mutations that predispose to hematologic malignancies, focusing on those most common during childhood and adolescence, based on the new International Consensus Classification of Myeloid and Lymphoid Neoplasms.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/05/16/mjhid-15-1-e2023032.PMC10171214.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9467183","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}
引用次数: 0
Gamma Heavy Chain Disease Associated with T-Cell Large Granular Lymphocyte Lymphoproliferative Disorder: Case Report and Literature Review. γ重链病与t细胞大颗粒淋巴细胞增生性疾病:病例报告和文献复习。
IF 3.2 4区 医学
Mediterranean Journal of Hematology and Infectious Diseases Pub Date : 2023-01-01 DOI: 10.4084/MJHID.2023.010
Maria Laura Bisegna, Maria Assunta Limongiello, Stefano Fiori, Irene Della Starza, Maria Stefania De Propris, Stefania Trasarti
{"title":"Gamma Heavy Chain Disease Associated with T-Cell Large Granular Lymphocyte Lymphoproliferative Disorder: Case Report and Literature Review.","authors":"Maria Laura Bisegna,&nbsp;Maria Assunta Limongiello,&nbsp;Stefano Fiori,&nbsp;Irene Della Starza,&nbsp;Maria Stefania De Propris,&nbsp;Stefania Trasarti","doi":"10.4084/MJHID.2023.010","DOIUrl":"https://doi.org/10.4084/MJHID.2023.010","url":null,"abstract":"Heavy chain diseases are rare B-cell neoplasms consisting of the production of a monoclonal immunoglobulin composed of the only heavy chain without corresponding light chains. It is a rare adult disease that may involve several sites with a variable clinical course. It manifests itself on a large spectrum from indolent to rapidly progressive. We present a case of heavy chain disease and concomitant T- cell large granular lymphoproliferative disorder, an association described in only six cases before.","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f4/a9/mjhid-15-1-e2023010.PMC9833300.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10551729","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}
引用次数: 0
Bacterial Infections in a Child with TD-β-thalassemia and Common Variable Immunodeficiency Due to a Novel NFKB1 Variant. 由一种新的NFKB1变异引起的TD-β-地中海贫血和常见可变免疫缺陷儿童的细菌感染
IF 3.2 4区 医学
Mediterranean Journal of Hematology and Infectious Diseases Pub Date : 2023-01-01 DOI: 10.4084/MJHID.2023.053
Wanting Li, Kun Yang, Jian Xiao, Xiaodong Liu
{"title":"Bacterial Infections in a Child with TD-β-thalassemia and Common Variable Immunodeficiency Due to a Novel <i>NFKB1</i> Variant.","authors":"Wanting Li,&nbsp;Kun Yang,&nbsp;Jian Xiao,&nbsp;Xiaodong Liu","doi":"10.4084/MJHID.2023.053","DOIUrl":"https://doi.org/10.4084/MJHID.2023.053","url":null,"abstract":"","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/81/60/mjhid-15-1-e2023053.PMC10497310.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10625943","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}
引用次数: 0
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