Mediterranean Journal of Hematology and Infectious Diseases最新文献

{"title":"CAR-T CELL THERAPY IN B-CELL ACUTE LYMPHOBLASTIC LEUKEMIA","authors":"Ugo Testa, Simona Sica, E. Pelosi, G. Castelli, Giuseppe Leone","doi":"10.4084/mjhid.2024.010","DOIUrl":"https://doi.org/10.4084/mjhid.2024.010","url":null,"abstract":"Treatment of refractory and relapsed (R/R) B acute lymphoblastic leukemia (B-ALL) is an unmet medical need in both children and adults. Studies carried out in the last two decades have shown that autologous T cells engineered to express a chimeric antigen receptor (CAR-T) represent an effective technique for treating these patients. Antigens expressed on B-cells, such as CD19, CD20, and CD22, represent targets suitable for treating patients with R/R B-ALL. CD19 CAR-T cells induce a high rate (80-90%) of complete remissions in both pediatric and adult R/R B-ALL patients. However, despite this impressive rate of responses, about half of responding patients relapse within 1-2 years after CAR-T cell therapy. Allo-HSCT after CAR-T cell therapy might consolidate the therapeutic efficacy of CAR-T and increase long-term outcomes; however, not all the studies that have adopted allo-HSCT as a consolidative treatment strategy have shown a benefit deriving from transplantation. For B-ALL patients who relapse early after allo-HSCT or those with insufficient T-cell numbers for an autologous approach, using T cells from the original stem cell donor offers the opportunity for the successful generation of CAR-T cells and for an effective therapeutic approach. Finally, recent studies have introduced allogeneic CAR-T cells generated from healthy donors or unmatched, which are opportunely manipulated with gene editing to reduce the risk of immunological incompatibility, with promising therapeutic effects. Keywords: CAR T; Acute Lymphoid Leukemia; Allogeneic CAR-T; Autologous CAR-T.","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139128303","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}

{"title":"ASSOCIATION BETWEEN LEUKEMIC EVOLUTION AND UNCOMMON CHROMOSOMAL ALTERATIONS IN PEDIATRIC MYELODYSPLASTIC SYNDROME","authors":"Viviane Lamim Lovatel, Beatriz Ferreira da Silva, Eliane Ferreira Rodrigues, Maria Luiza Rocha da Rosa Borges, Rita de Cássia Barbosa Tavares, Ana Paula Silva Bueno, Elaine Sobral da Costa, Terezinha De Jesus Marques, T. Fernandez","doi":"10.4084/mjhid.2024.003","DOIUrl":"https://doi.org/10.4084/mjhid.2024.003","url":null,"abstract":"Background and objective: Pediatric myelodysplastic syndrome (pMDS) is a group of rare clonal neoplasms with a difficult diagnosis and risk of progression to acute myeloid leukemia (AML). The early stratification in risk groups is essential to choosing the treatment and indication for allogeneic hematopoietic stem cell transplantation (HSCT). According to the Revised International Prognostic Scoring System, cytogenetic analysis has demonstrated an essential role in diagnosis and prognosis. In pMDS, abnormal karyotypes are present in 30-50% of the cases.  Monosomy 7 is the most common chromosomal alteration associated with poor prognosis. However, the rarity of specific cytogenetic alterations makes its prognosis uncertain. Thus, this study aimed to describe uncommon cytogenetic alterations in a cohort of 200 pMDS patients and their association with evolution to AML. Methods: The cytogenetic analysis was performed in 200 pMDS patients by G-banding and fluorescence in situ hybridization between 2000 to 2022. Results: Rare chromosome alterations were observed in 7.5% (15/200) of the cases. These chromosome alterations were divided into four cytogenetic groups: hyperdiploidy, biclonal chromosomal alterations, translocations, and uncommon deletions, which represented 33.3%, 33.3%, 20%, and 13.3%, respectively. Most of these patients (10/15) were classified with advanced MDS (MDS-EB and MDS/AML) and the initial subtype was present in five patients (RCC). The leukemic evolution was observed in 66.66% (10/15) of the patients. Most patients had poor clinical outcomes and they were indicated for HSCT.  Conclusion: The study of uncommon cytogenetic alterations in pMDS is important to improve the prognosis and guide early indication of HSCT.  Keywords: Pediatric MDS; Leukemic evolution; rare chromosomal altwerations; HSCT, Children","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139125809","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}

{"title":"A rare immune-related mesenteric and retroperitoneal serositis in a multirefractory Hodgkin lymphoma patient successfully treated with chylolymphostatic surgery","authors":"Matteo D'Addona, Luca Pezzullo, Corradino Campisi, Corrado Cesare Campisi, Valentina Giudice, Vincenzo Ciccone, Roberto Guariglia, Bianca Serio, Carmine Selleri","doi":"10.4084/mjhid.2023.063","DOIUrl":"https://doi.org/10.4084/mjhid.2023.063","url":null,"abstract":"","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":"136069796","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}

{"title":"Impact of Sars-CoV-2 prophylaxis with tixagevimab-cilgavimab in high-risk patients with B-cell malignancies: a single-center retrospective study.","authors":"Giovanni Manfredi Assanto ,, Matteo Totaro, Poggiali Rebecca, Delli Paoli Adele, Annechini Giorgia, D’Elia Gianna Maria, Aji Francesco, Petrucci Luigi, Fazio Francesca, Del Giudice Ilaria, Martelli Maurizio, Micozzi Alessandra, Giuseppe Gentile","doi":"10.4084/mjhid.2023.061","DOIUrl":"https://doi.org/10.4084/mjhid.2023.061","url":null,"abstract":"Severe Acute Respiratory Syndrome CoronaVirus‐2 (SARS‐CoV‐2) infection can result in different clinical manifestations (COVID-19), starting from asymptomatic disease to life threatening respiratory insufficiency. Onco-haematologic patients are at higher risk to develop severe COVID-19. In particular, patients affected by lymphoproliferative diseases, given the impaired cell-mediated and antibody-mediated immunity and treatment toxicity, develop more often a symptomatic and a more serious disease of Covid-19. Various therapeutic and prophylactic agents are being used against COVID‐19 such as antiviral drugs, vaccines and antiviral S‐protein monoclonal antibodies. Pre-exposure prophylaxis with AZD442/Evusheld (tixagevimab-cilgavimab) may be a complementary strategy to decrease the incidence or severity of COVID-19 for patients with B-cell malignancies. Tixagevimab-cilgavimab is a combination of two monoclonal antibodies that bind SARS-CoV-2 spike protein and inhibits the attachment to the surface of cells, preventing viral entry in the cell and COVID-19 development. In the setting of hematology real-life, few data are available on the impact of pre-exposure prophylaxis, given the multiple factors involved in the clinical behavior of SARS-CoV-2 . Our aim was to evaluate the clinical benefit and the safety of this strategy at our center.","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":"136104529","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}

{"title":"A novel ALAS2 mutation causes congenital sideroblastic anemia","authors":"Kun Yang","doi":"10.4084/mjhid.2023.062","DOIUrl":"https://doi.org/10.4084/mjhid.2023.062","url":null,"abstract":"","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":"136068972","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}

{"title":"KINETICS AND ROLE OF PANCREATIC STONE PROTEIN AND MIDREGIONAL PROADRENOMEDULLIN AS PREDICTORS OF SEPSIS AND BACTEREMIA IN CHILDREN WITH HAEMATOLOGICAL MALIGNANCIES.","authors":"Vasiliki Antari, Lemonia Skoura, Athanasios Tragiannidis, Emmanuel Hatzipantelis, Vasiliki-Regina Tsinopoulou, Konstantina Papakonstantinou, Efthimia Protonotariou, Assimina Galli-Tsinopoulou","doi":"10.4084/mjhid.2023.065","DOIUrl":"https://doi.org/10.4084/mjhid.2023.065","url":null,"abstract":"Background: To investigate the kinetics and prognostic value of pancreatic stone protein (PSP) and mid-regional proadrenomedullin (MR-proADM) during episodes of febrile neutropenia (FN) in children with haematological malignancies.
 Material and methods: We evaluated prospectively a total of 70 FN episodes in 70 children with acute leukemias and lymphomas. Levels of CRP, PSP and MR-proADM were measured at the onset of the febrile episode (day 1), on day 3 and on day 7. The outcome and survival of children were evaluated during the study period until day 28. The performance of each marker in identifying sepsis or severe sepsis was assessed as area under a receiver operating characteristic (ROC) curve. For each biomarker, ROC curves were used to derive cut-offs for sensitivity and specificity in distinguishing sepsis from non-sepsis.
 Results: During the 2-year study period, 70 febrile neutropenia episodes in 70 children with haematological malignancies were enrolled. Of 70 episodes of febrile neutropenia, in 17 (24%) a bacterial/fungal infection was documented. Criteria for sepsis were fulfilled for 31 (44%) and 7 (10%) patients were admitted to PICU. The median values of all biomarkers were higher on day 1 and differed significantly between patients with and without sepsis. The specificity of PSP, MR-proADM, and CRP were 0.82, 0.70, and 0.57, respectively. The sensitivity of PSP, MR-proADM and CRP were 0.84, 0.74, and 0.88, respectively.
 Conclusions: Both PSP and MR-proADM are promising biomarkers for early diagnosis of sepsis during FN episodes in children with haematological malignancies. PSP has the higher sensitivity and specificity.","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":"136068976","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}

{"title":"LIVER DISEASE & SICKLE CELL DISEASE: AUTOIMMUNE HEPATITIS MORE THAN A COINCIDENCE; A SYSTEMATIC LITERATURE REVIEW.","authors":"Kelvin Lynch, Andrea Mega, Andrea Piccin, Massimo Daves, Helen Fogarty","doi":"10.4084/mjhid.2023.060","DOIUrl":"https://doi.org/10.4084/mjhid.2023.060","url":null,"abstract":"In patients with SCD, chronic liver damage is a common manifestation. More than 50% of SCD patients have elevated liver enzymes. Common underlying aetiologies include sickle cell hepatic crisis, viral hepatitis, sickle cell intrahepatic cholestasis and hepatic sequestration in the acute setting, and cholelithiasis and iron overload in the chronic setting. Autoimmune hepatitis (AIH) is a rare disease that appears to occur more commonly in the sickle cell disease (SCD) population than in the general population. There are many schools of thought as to why this is the case, including the phosphatidylserine hypothesis, the heme inflammatory hypothesis, the complement generation hypothesis, and the transfusion alloimmunization hypothesis. Due to the natural history of the two illnesses, SCD is almost always diagnosed first in cases of dual pathology. Symptoms such as jaundice, fatigue, and abdominal pain are common in SCD, as are abnormal liver function tests (LFTs). These abnormalities, attributed to the other more frequent liver involvements in SCD, can lead to delays in AIH diagnosis in this population. Corticosteroids, sometimes with other immunosuppressive agents, such as azathioprine, are the cornerstone of acute AIH treatment (4). However, corticosteroid use in the SCD population has been shown to carry an increased risk of vaso-occlusive crises (1, 5), providing a treatment dilemma. The following is a review of AIH in the SCD population, where we explore the pathophysiology behind the association between the two disorders, discuss an approach to investigating abnormal LFTs in SCD, and examine treatment options in this population with co-existing diseases.","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":"136069586","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}

{"title":"Diagnosis and prognosis evaluation of severe pneumonia by lung ultrasound score combined with serum inflammatory markers","authors":"Bo Fu, Peng Zhang, JunHua Zhang","doi":"10.4084/mjhid.2023.057","DOIUrl":"https://doi.org/10.4084/mjhid.2023.057","url":null,"abstract":"Objective: To analyze the significance of lung ultrasound score (LUS) combined with serum inflammatory indexes in different severities of severe pneumonia and and its clinical value on prognosis.
 Methods: A total of 100 patients with severe pneumonia who were treated in the Gansu Provincial Hospital from June 2017 to June 2021 were selected as the research objects. According to the acute physiology and chronic health (APACHE II) score, they were divided into low-risk group (28 cases) and medium-risk group (39 cases) and high-risk group (33 cases). The general clinical data of the patients (age, gender, smoking history, and underlying diseases) were collected, the lung ultrasound score (LUS) of the patients was measured, and the serum inflammatory indicators (IL-6, IL-10, TNF-α, CRP and NLR) levels; pearson correlation analysis to evaluate the correlation between LUS score, serum inflammatory index levels and disease severity; receiver operating characteristic (ROC) curve analysis to evaluate the prognostic value of the combined diagnosis of LUS score and serum inflammatory index for the severity of severe pneumonia.
 Results: With the increase of the severity of severe pneumonia, the LUS score and the level of inflammation in the body continued to increase, and LUS combined with serum inflammatory indexes could distinguish the severity of low-risk, medium-risk and high-risk of severe pneumonia, and had high diagnostic value. In addition, the combined diagnosis of LUS and serum inflammatory markers is also closely related to the prognosis of patients with severe pneumonia, which can distinguish the prognosis.
 Conclusion: LUS combined with serum inflammatory indicators (IL-6, IL-10, TNF-α, CRP and NLR) can differentiate the severity and prognosis of severe pneumonia, which may be a new direction for the diagnosis of severe pneumonia and guide early clinical intervention.","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":"136023232","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}

{"title":"Evaluating the use of meropenem in hematologic patients with febrile neutropenia","authors":"R.A. Stuurman, E. Jong, P.C.R. Godschalk, M.F. Corsten, J.E. Nagtegaal","doi":"10.4084/mjhid.2023.067","DOIUrl":"https://doi.org/10.4084/mjhid.2023.067","url":null,"abstract":"Abstract
 Objectives 
 The Antibiotic Stewardship Team of Meander Medical Centre (Meander MC) instigated a revaluation of its treatment protocol for hematologic patients admitted with febrile neutropenia. The current hospital protocol advises administering meropenem for 72 hours, followed by antibiotic therapy guided by microbiological cultures. In order to responsibly adjust the current empiric regimen, this study aimed to determine the frequency of bacteria resistant to alternative antibiotics, namely ceftazidime and piperacillin/tazobactam, in both surveillance and diagnostic cultures.
 Methods
 This retrospective, observational, single-centre study included adult patients with a hematologic malignancy and febrile neutropenia admitted between October 2018 and June 2021. Collected metadata included patient characteristics, surveillance and diagnostic culture results, and antibiotic use.
 Results
 A total of 100 patients were included. One or more bacteria resistant to ceftazidime or piperacillin/tazobactam were identified in blood and urine cultures in seven (7%) and one (1%) patients respectively.
 Conclusions
 Our results support the safe reduction of the use of meropenem by changing the empiric treatment protocol for patients with hematologic malignancy and febrile neutropenia. As this study showed a lower resistance frequency to piperacillin/tazobactam than to ceftazidime, this antibiotic is the recommended alternative.","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":"136103423","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}

{"title":"THERAPY-RELATED MYELOID NEOPLASMS: PREDISPOSITION AND CLONAL EVOLUTION","authors":"Emiliano Fabiani, Giulia Falconi, Antonio Cristiano, H. Hajrullaj, Giulia Falconi, Giuseppe Leone, Maria Teresa Voso","doi":"10.4084/mjhid.2023.064","DOIUrl":"https://doi.org/10.4084/mjhid.2023.064","url":null,"abstract":"Therapy-related Myeloid Neoplasm (t-MN) represents one of the worst long-term consequences of cytotoxic therapy for primary tumors and autoimmune disease. Poor survival and refractoriness to current treatment strategies characterize affected patients from a clinical point of view. In our aging societies, where newer therapies and ameliorated cancer management protocols are improving the life expectancy of cancer patients, therapy-related Myeloid Neoplasms are an emerging problem. Although several research groups have contributed to characterizing the main risk factors in t-MN development, the multiplicity of primary tumors, in association with the different therapeutic strategies available and the new drugs in development, make interpreting the current data still complex. The main risk factors involved in t-MN pathogenesis can be subgrouped into patient-specific, inherited, and acquired predispositions. Although t-MN can occur at any age, the risk tends to increase with advancing age, and older patients, characterized by a higher number of comorbidities, are more likely to develop the disease. Thanks to the availability of deep sequencing techniques, germline variants have been reported in 15-20% of t-MN patients, highlighting their role in cancer predisposition. It is becoming increasingly evident that t-MN with driver gene mutations may arise in the background of Clonal Hematopoiesis of Indeterminate Potential (CHIP) under the positive selective pressure of chemo and/or radiation therapies. Although CHIP is generally considered benign, it has been associated with an increased risk of t-MN. In this context, the phenomenon of clonal evolution may be described as a dynamic process of expansion of preexisting clones, with or without acquisition of additional genetic alterations, that, by favoring the proliferation of more aggressive and/or resistant clones, may play a crucial role in the progression from preleukemic states to t-MN.","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":"136068971","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}