Mediterranean Journal of Hematology and Infectious Diseases最新文献

{"title":"Rituximab versus Splenectomy in Chronic Primary ITP: Experience of a Single Hematology Clinic.","authors":"Rawand Polus Shamoon, Ahmed Khudair Yassin, Sarah Laith Alnuaimy","doi":"10.4084/MJHID.2024.019","DOIUrl":"10.4084/MJHID.2024.019","url":null,"abstract":"<p><strong>Background: </strong>Immune thrombocytopenia (ITP) is an acquired immune-mediated disease that lacks an underlying etiology. Steroids are the main first-line treatment of ITP, while the second-line treatment consists primarily of splenectomy and rituximab. This study aimed to assess and compare the response to rituximab and splenectomy.</p><p><strong>Methods: </strong>This retrospective comparative study reviewed ITP patients treated at a single private hematology clinic from 2007 to 2019. Seventy-four ITP patients were recruited, 27 were on rituximab, and 47 had undergone splenectomy. The initial platelet counts and bleeding symptoms were recorded, and initial and long-term responses to treatment were evaluated based on the American Society of Hematology guidelines.</p><p><strong>Results: </strong>The mean age of the patients was 42.1 years with a male-to-female ratio of 1:1.8. The initial mean platelet count was comparable between the rituximab and splenectomy groups (p = 0.749). The initial complete response (CR) differed significantly between the rituximab and splenectomy groups (44.4% versus 83%, p = 0.002). The five-year response rate was significantly higher in the splenectomy than in the rituximab group (74% versus 52%, log-rank 0.038). Splenectomy was the only significant predictive factor for long-term response (OR = 0.193, p = 0.006).</p><p><strong>Conclusion: </strong>The overall response revealed that splenectomy appeared superior to rituximab as a second-line treatment of ITP. Splenectomy was the only positive prognostic indicator of sustained response.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10927223/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140101996","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":"Impact of Daratumumab on Stem Cell Mobilization and Transplant in Patient with Newly Diagnosed Multiple Myeloma: A Real Word Single-Centre Study.","authors":"Mauro Passucci, Francesca Fazio, Jacopo Micozzi, Manhaz Shafii Bafti, Giovanni Assanto, Alfonso Piciocchi, Maurizio Martelli, Maria Teresa Petrucci","doi":"10.4084/MJHID.2024.041","DOIUrl":"10.4084/MJHID.2024.041","url":null,"abstract":"","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11178051/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141332911","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":"Coexistence of Multiple Gene Variants in Some Patients with Erythrocytoses.","authors":"Andrea Benetti, Irene Bertozzi, Giulio Ceolotto, Irene Cortella, Daniela Regazzo, Giacomo Biagetti, Elisabetta Cosi, Maria Luigia Randi","doi":"10.4084/MJHID.2024.021","DOIUrl":"10.4084/MJHID.2024.021","url":null,"abstract":"<p><strong>Background: </strong>Erythrocytosis is a relatively common condition; however, a large proportion of these patients (70%) remain without a clear etiologic explanation.</p><p><strong>Methods: </strong>We set up a targeted NGS panel for patients with erythrocytosis, and 118 sporadic patients with idiopathic erythrocytosis were studied.</p><p><strong>Results: </strong>In 40 (34%) patients, no variant was found, while in 78 (66%), we identified at least one germinal variant; 55 patients (70.5%) had 1 altered gene, 18 (23%) had 2 alterations, and 5 (6.4%) had 3. An altered <i>HFE</i> gene was observed in 51 cases (57.1%), <i>EGLN1</i> in 18 (22.6%) and <i>EPAS1</i>, <i>EPOR</i>, <i>JAK2</i>, and <i>TFR2</i> variants in 7.7%, 10.3%, 11.5%, and 14.1% patients, respectively. In 23 patients (19.45%), more than 1 putative variant was found in multiple genes.</p><p><strong>Conclusions: </strong>Genetic variants in patients with erythrocytosis were detected in about 2/3 of our cohort. An NGS panel including more candidate genes should reduce the number of cases diagnosed as \"idiopathic\" erythrocytosis in which a cause cannot yet be identified. It is known that <i>HFE</i> variants are common in idiopathic erythrocytosis. <i>TFR2</i> alterations support the existence of a relationship between genes involved in iron metabolism and impaired erythropoiesis. Some novel multiple variants were identified. Erythrocytosis appears to be often multigenic.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10927185/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140101961","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":"NG2 Molecule Expression in Acute Lymphoblastic Leukemia B Cells: A Flow-Cytometric Marker for the Rapid Identification of <i>KMT2A</i> Gene Rearrangements.","authors":"Maria Laura Bisegna, Nadia Peragine, Loredana Elia, Mabel Matarazzo, Maria Laura Milani, Stefania Intoppa, Mariangela Di Trani, Francesco Malfona, Maurizio Martelli, Maria Stefania De Propris","doi":"10.4084/MJHID.2024.018","DOIUrl":"10.4084/MJHID.2024.018","url":null,"abstract":"<p><strong>Background: </strong>B-lineage acute lymphoblastic leukemias (B-ALL) harboring rearrangements of the histone lysine [K]-Methyltransferase 2A (<i>KMT2A</i>) gene on chromosome 11q23 (<i>KMT2A-r</i>) represent a category with dismal prognosis. The prompt identification of these cases represents an urgent clinical need. Considering the correlation between rat neuron glial-antigen 2 (NG2) chondroitin-sulfate-proteoglycan molecule expression and <i>KMT2A-r</i>, we aimed to identify an optimized cytofluorimetric diagnostic panel to predict the presence of <i>KMT2A-r</i>.</p><p><strong>Materials and methods: </strong>We evaluated 88 NG2+ B-ALL cases identified with an NG2 positivity threshold >10% from a cohort of 1382 newly diagnosed B-ALLs referred to the Division of Hematology of 'Sapienza' University of Rome.</p><p><strong>Results: </strong>Eighty-five of 88 (96.6%) NG2+ B-ALLs harbored <i>KMT2A-r</i> and were mainly pro-B ALL (77/85; 91%). Only 2 B-ALLs with <i>KMT2A-r</i> showed NG2 expression below 10%, probably due to the steroid therapy administered prior to cytofluorimetric analysis.Compared to <i>KMT2A-r-</i>cases, <i>KMT2A r+</i> B-ALLs showed a higher blast percentage, significantly higher mean fluorescence intensity (MFI) of CD45, CD38, and CD58, and significantly lower MFI of CD34, CD22, TdT, and CD123.The study confirmed differences in CD45, CD34, CD22, and TdT MFI within the same immunologic EGIL group (European Group for the immunological classification of leukemias), indicating no influence of the B-ALLs EGIL subtype on the <i>KMT2A-r+</i> B-ALLs immunophenotype.</p><p><strong>Conclusions: </strong>Our data demonstrate the association between NG2 and <i>KMT2A-r</i> in B-ALLs identify a distinctive immunophenotypic pattern, useful for rapid identification in diagnostic routines of these subtypes of B-ALLs with a poor prognosis that benefits from a specific therapeutic approach.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10927233/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140101968","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":"Clinical Signs and Treatment of New-Onset Bone Marrow Failure Associated SARS-CoV-2 Infection in Children: A Single Institution Prospective Cohort Study.","authors":"Mervat A M Youssef, Ebtisam Shawky Ahmed, Dalia Tarik Kamal, Khalid I Elsayh, Mai A Abdelfattah, Hyam Hassan Mahran, Mostafa M Embaby","doi":"10.4084/MJHID.2024.034","DOIUrl":"10.4084/MJHID.2024.034","url":null,"abstract":"<p><strong>Background: </strong>Viral infections can cause direct and indirect damage to hematopoietic stem cells. The objectives of this study were to identify the frequency and severity of aplastic anemia in children infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) as well as recognize the response to treatment.</p><p><strong>Methodology: </strong>13 children with newly diagnosed severe aplastic anemia were enrolled in this prospective clinical trial. Blood samples were obtained from all patients to detect SARS-CoV-2 antibodies, and nasopharyngeal swabs were collected for reverse-transcription Polymerase Chain Reaction to detect SARS-CoV-2 viruses. According to the laboratory results, patients were classified as having SARS-CoV-2 positive antibodies and SARS-CoV-2 negative antibodies. Both groups received combined cyclosporine (CsA) + Eltrombopag (E-PAG). The hematological response, either complete response (CR) or partial response (PR), no response (NR), and overall response (OR) rates of combined E-PAG + CsA treatment after 6 months were evaluated.</p><p><strong>Results: </strong>Four children were recognized to have aplastic anemia and SARS-CoV-2 positive antibodies. Two patients fulfilled the hematological criteria for CR and no longer required transfusion of packed red blood cells (PRBCs) or platelets, and one had PR and was still PRBC transfusion-dependent but no longer required platelet transfusion. The remaining patient showed NR, and he had died before reaching the top of the HSCT waiting list. Moreover, six patients in the SARS-CoV-2 negative antibodies group had CR, while three patients had PR. The difference in ANC, Hg, and platelet counts between both groups was not significant.</p><p><strong>Conclusion: </strong>The SARS-CoV-2 virus is added to several viral infections known to be implicated in the pathogenesis of aplastic anemia. Studies are needed to establish a definitive association and determine whether the response of bone marrow failure to standard therapy differs from that of idiopathic cases.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10927230/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140101960","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":"Comparison of Infectious Complications in Patients Receiving High-Dose Cyclophosphamide as GvHD Prophylaxis After Transplantation From A 9/10 HLA-Matched Unrelated Donor with Standard GvHD Prophylaxis After Transplant From A Fully Matched Related Donor.","authors":"Selim Sayýn, Murat Yýldýrým, Melda Cömert, Bilge Uğur, Esra Şafak Yýlmaz, Ferit Avcu, Ali Uğur Ural, Meltem Aylı","doi":"10.4084/MJHID.2024.016","DOIUrl":"10.4084/MJHID.2024.016","url":null,"abstract":"<p><strong>Background: </strong>The aim of this study was to evaluate whether cyclophosphamide administered after allogeneic stem cell transplantation (ASCT) from 9/10 HLA-Matched Unrelated Donors (MMUD) increases the rates of bacterial, fungal, viral infections, complications (hemorrhagic cystitis (HC)), and infection-related mortality compared to allogeneic stem cell transplantation from matched related donors (MRD).</p><p><strong>Methods: </strong>This is a retrospective multicenter study. 45 MMUD ASCT patients who received posttransplant cyclophosphamide+methotrexate+calcineurin inhibitor compared with 45 MRD ASCT patients who received methotrexate+calcineurin inhibitor.</p><p><strong>Results: </strong>Although there was a statistically significant prolongation of neutrophil engraftment time in the PTCy arm, there was no statistically significant difference in bacterial infection frequencies between the groups (PTCy; 9 (20%), control; 8 (17.8%), p=0.778). The distribution of CMV infection in the first 100 days was similar (p=0.827), but the distribution of CMV infection rate between the 100th and 365th days was observed more frequently in the control group (p=0.005). HC rates and their grades were similar in both groups (PTCy; 4 (8.8%), control; 6 (13.3%) p=0.502). The rates of VZV infection and invasive aspergillosis were similar in the PTCy and control groups (13.3% in the PTCy and 17.8% in the control group p=0.561). There is also no statistically significant difference in survival analysis (OS, LFS, GRFS, RI, IRM, NRM) between groups. However, the incidence of cGVHD was significantly higher in the control group (P=0.035).</p><p><strong>Conclusions: </strong>The addition of PTCy to standard GvHD prophylaxis in MMUD ASCT does not lead to an increase in CMV reactivation, bacterial infections, invasive fungal infection, viral hemorrhagic cystitis, or mortality.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10927186/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140101962","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":"Antibody Response to Breakthrough SARS-CoV-2 Infection in \"Booster\" Vaccinated Patients with Multiple Myeloma According to B/T/NK Lymphocyte Absolute Counts and anti-CD38 Treatments.","authors":"Nicola Sgherza, Anna Mestice, Angela Maria Vittoria Larocca, Pellegrino Musto","doi":"10.4084/MJHID.2024.022","DOIUrl":"10.4084/MJHID.2024.022","url":null,"abstract":"","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10927213/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140103167","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":"Successful Treatment of De Novo Acute Myeloid Leukemia-Associated Aortitis by Induction Chemotherapy Alone.","authors":"Urbain Tauveron-Jalenques, Vincent Grobost, Benoît Magnin, Cécile Moluçon-Chabrot, Jacques-Olivier Bay, Olivier Tournilhac, Romain Guièze","doi":"10.4084/MJHID.2024.025","DOIUrl":"10.4084/MJHID.2024.025","url":null,"abstract":"","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10927218/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140101998","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":"Internal Medicine Ward with Hematological Skills for the Treatment of Complications Suffered by Hematological Patients on Therapy: Experience of Villa Betania Hospital in Rome.","authors":"A Andriani, L Marchetti, F Rossi, M A Perretti, S Raja, U Recine","doi":"10.4084/MJHID.2024.030","DOIUrl":"10.4084/MJHID.2024.030","url":null,"abstract":"","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10927202/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140101967","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":"Plasmablastic Lymphoma. A State-of-the-Art Review: Part 2-Focus on Therapy.","authors":"Michele Bibas","doi":"10.4084/MJHID.2024.015","DOIUrl":"10.4084/MJHID.2024.015","url":null,"abstract":"<p><p>The objective of this two-part review is to present a current and comprehensive understanding of the diagnosis and management of plasmablastic lymphoma. The first part, which was published previously, focused on the study of epidemiology, etiology, clinicopathological characteristics, differential diagnosis, prognostic variables, and the impact of plasmablastic lymphoma on specific populations. This second part addresses the difficult topic of the treatment of plasmablastic lymphoma, specifically examining both the conventional, consolidated approach and the novel therapeutic strategy.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10927196/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140101969","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}