The objective of this two-part study is to present current and comprehensive understanding on the diagnosis and management of plasmablastic lymphoma. The first section, as presented in this paper, is on the study of epidemiology, etiology, clinopathological characteristics, differential diagnosis, prognostic variables, and the impact of plasmablastic lymphoma on specific populations. Plasmablastic lymphoma (PBL), a rare and aggressive form of lymphoma. Previous and modern studies have demonstrated a significant association between the human immunodeficiency virus (HIV) and the development of the disease. The limited occurrence of PBL contributes to a lack of comprehensive understanding regarding the molecular mechanisms involved in its etiology. Consequently, the diagnostic procedure for PBL poses a significant difficulty. Among the group of CD20-negative large B-cell lymphomas, PBL can be correctly diagnosed by identifying its exact clinical characteristics, anatomical location, and morphological characteristics. PBL cells do not express CD20 or PAX5 but possess plasmacytic differentiation markers such as CD38, CD138, MUM1/IRF4, Blimp1, and XBP1. PBL must be distinguished from other B-cell malignancies that lack the CD20 marker, including primary effusion lymphoma, anaplastic lymphoma kinase-positive large B-cell lymphoma, and large B-cell lymphoma (LBCL). This condition is frequently associated with infections caused by the Epstein-Barr virus and genetic alterations involving the MYC gene. Despite advances in our comprehension of this disease, the prognosis remains dismal, resulting in a low overall survival rate, although recent reports suggest an apparent tendency towards substantial improvement.
本研究由两部分组成，旨在介绍目前对浆细胞性淋巴瘤诊断和管理的全面认识。本文的第一部分主要研究浆细胞性淋巴瘤的流行病学、病因学、临床病理学特征、鉴别诊断、预后变量以及对特定人群的影响。 浆细胞性淋巴瘤（PBL）是一种罕见的侵袭性淋巴瘤。以往和现代的研究都表明，人类免疫缺陷病毒（HIV）与该病的发生有显著关联。由于 PBL 的发病率有限，因此对其病因的分子机制缺乏全面的了解。因此，PBL 的诊断程序存在很大困难。在 CD20 阴性大 B 细胞淋巴瘤中，PBL 可通过确定其确切的临床特征、解剖位置和形态学特征来正确诊断。PBL 细胞不表达 CD20 或 PAX5，但具有浆细胞分化标记，如 CD38、CD138、MUM1/IRF4、Blimp1 和 XBP1。PBL 必须与其他缺乏 CD20 标记的 B 细胞恶性肿瘤区分开来，包括原发性渗出淋巴瘤、无性淋巴瘤激酶阳性大 B 细胞淋巴瘤和大 B 细胞淋巴瘤（LBCL）。这种疾病通常与 Epstein-Barr 病毒感染和涉及 MYC 基因的遗传改变有关。尽管我们对这种疾病的认识有所进步，但其预后仍然不容乐观，总体存活率很低，不过最近的报告显示，这种疾病有明显好转的趋势。
{"title":"PLASMABLASTIC LYMPHOMA. A STATE-OF-THE-ART REVIEW (1)","authors":"Michele Bibas","doi":"10.4084/mjhid.2024.007","DOIUrl":"https://doi.org/10.4084/mjhid.2024.007","url":null,"abstract":"The objective of this two-part study is to present current and comprehensive understanding on the diagnosis and management of plasmablastic lymphoma. The first section, as presented in this paper, is on the study of epidemiology, etiology, clinopathological characteristics, differential diagnosis, prognostic variables, and the impact of plasmablastic lymphoma on specific populations. Plasmablastic lymphoma (PBL), a rare and aggressive form of lymphoma. Previous and modern studies have demonstrated a significant association between the human immunodeficiency virus (HIV) and the development of the disease. The limited occurrence of PBL contributes to a lack of comprehensive understanding regarding the molecular mechanisms involved in its etiology. Consequently, the diagnostic procedure for PBL poses a significant difficulty. Among the group of CD20-negative large B-cell lymphomas, PBL can be correctly diagnosed by identifying its exact clinical characteristics, anatomical location, and morphological characteristics. PBL cells do not express CD20 or PAX5 but possess plasmacytic differentiation markers such as CD38, CD138, MUM1/IRF4, Blimp1, and XBP1. PBL must be distinguished from other B-cell malignancies that lack the CD20 marker, including primary effusion lymphoma, anaplastic lymphoma kinase-positive large B-cell lymphoma, and large B-cell lymphoma (LBCL). This condition is frequently associated with infections caused by the Epstein-Barr virus and genetic alterations involving the MYC gene. Despite advances in our comprehension of this disease, the prognosis remains dismal, resulting in a low overall survival rate, although recent reports suggest an apparent tendency towards substantial improvement.","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"50 28","pages":""},"PeriodicalIF":3.2,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139125469","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}
Thalidomide is a therapeutic option for patients with β-thalassemia by increasing fetal hemoglobin and thereby reducing the requirement for blood transfusions. However, information on changes in erythropoiesis and iron homeostasis during thalidomide treatment is lacking. This study investigated the effects of thalidomide treatment on hematologic, erythropoietic, and iron-status parameters in 22 patients with transfusion-dependent β-thalassemia (TDT). Thalidomide significantly improved anemia endpoints, including increases in hemoglobin (p<0.001), red blood cells (p<0.001), and hematocrit (p<0.001), as well as reducing erythropoietin levels (p=0.033) and ameliorating erythropoiesis. Thalidomide treatment significantly reduced serum iron levels (p=0.018) and transferrin saturation (p=0.039) and increased serum transferrin levels (p=0.030). Thalidomide had no observed effect on serum ferritin or hepcidin, but changes in hepcidin(r=0.439, p=0.041) and serum iron (r=−0.536, p=0.010) were significantly correlated with hemoglobin increment. This comprehensive study indicates that thalidomide treatment can ameliorate erythropoiesis and iron homeostasis in patients with TDT, thus supporting the effectiveness of this drug.
{"title":"THALIDOMIDE AMELIORATES ERYTHROPOIESIS AND IRON HOMEOSTASIS IN TRANSFUSION-DEPENDENT β-THALASSEMIA","authors":"Kun Yang, Jian Xiao","doi":"10.4084/mjhid.2024.001","DOIUrl":"https://doi.org/10.4084/mjhid.2024.001","url":null,"abstract":"Thalidomide is a therapeutic option for patients with β-thalassemia by increasing fetal hemoglobin and thereby reducing the requirement for blood transfusions. However, information on changes in erythropoiesis and iron homeostasis during thalidomide treatment is lacking. This study investigated the effects of thalidomide treatment on hematologic, erythropoietic, and iron-status parameters in 22 patients with transfusion-dependent β-thalassemia (TDT). Thalidomide significantly improved anemia endpoints, including increases in hemoglobin (p<0.001), red blood cells (p<0.001), and hematocrit (p<0.001), as well as reducing erythropoietin levels (p=0.033) and ameliorating erythropoiesis. Thalidomide treatment significantly reduced serum iron levels (p=0.018) and transferrin saturation (p=0.039) and increased serum transferrin levels (p=0.030). Thalidomide had no observed effect on serum ferritin or hepcidin, but changes in hepcidin(r=0.439, p=0.041) and serum iron (r=−0.536, p=0.010) were significantly correlated with hemoglobin increment. This comprehensive study indicates that thalidomide treatment can ameliorate erythropoiesis and iron homeostasis in patients with TDT, thus supporting the effectiveness of this drug.","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"41 24","pages":""},"PeriodicalIF":3.2,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139125608","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}
Introduction : According to WHO, regular, voluntary, unpaid blood donors are the safest group of donors, as they have the lowest prevalence of blood transmitted infections. However, family/replacement blood donors is widely used in sub Saharan Africa and this practice was exacerbated during the COVID 19 pandemic. This study aimed to compare the seroprevalence of infectious markers in family replacement blood donors and voluntary non-remunerated blood donors during the COVID 19 pandemic in a country of sub Saharan Africa. Materials and Methods Blood donors received at the National Centre of Blood Transfusion (NBTC) of Dakar from August 1st to October 31th 2021, were included in this study. All donors completed a pre-donation questionnaire. Donors identity, epidemiological parameters, reasons for donation and laboratory results were collected in the Inlog® software of the NBTC. The serological tests for HBV, HIV and HCV were performed with chemiluminescence technology. The Rapid Plasma Reagent test was used to find out treponemal antibodies. The determination of ABO and Rh blood groups was performed using monoclonal antisera following classical hemagglutination test on a plate. Results A total of 5002 donors were collected during this COVID-19 pandemic period. Blood family/replacement donors represented 54.0% and new voluntary donors represented 52.6%. Comparison of HIV, HCV and syphilis markers seroprevalence showed no statistically significant difference between new voluntary donors and new family replacement donors (p>0.05). However, for HBV the seroprevalence was significantly higher in new family replacement donors (p=0,002). Conclusion The proper supply of blood was impacted by the COVID-19 pandemic meanwhile replacement donations had contributed to limiting the damage observed with blood shortages. However, the significant differences noted on the seroprevalences of transfusion-transmissible infections between voluntary non-paid donors and family/replacement donors strengthens WHO recommendations for the selection of volunteer non-paid donors to lower transfusion-transmissible HBV in sub Saharan Africa.
{"title":"Seroprevalence of transfusion-transmissible infections among family replacement donors and voluntary non-remunerated blood donors during the COVID-19 pandemic in sub Saharan Africa","authors":"M. Gadji, Y. Guéye, David Motto, Saliou Diop","doi":"10.4084/mjhid.2024.008","DOIUrl":"https://doi.org/10.4084/mjhid.2024.008","url":null,"abstract":"Introduction : According to WHO, regular, voluntary, unpaid blood donors are the safest group of donors, as they have the lowest prevalence of blood transmitted infections. However, family/replacement blood donors is widely used in sub Saharan Africa and this practice was exacerbated during the COVID 19 pandemic. This study aimed to compare the seroprevalence of infectious markers in family replacement blood donors and voluntary non-remunerated blood donors during the COVID 19 pandemic in a country of sub Saharan Africa. Materials and Methods Blood donors received at the National Centre of Blood Transfusion (NBTC) of Dakar from August 1st to October 31th 2021, were included in this study. All donors completed a pre-donation questionnaire. Donors identity, epidemiological parameters, reasons for donation and laboratory results were collected in the Inlog® software of the NBTC. The serological tests for HBV, HIV and HCV were performed with chemiluminescence technology. The Rapid Plasma Reagent test was used to find out treponemal antibodies. The determination of ABO and Rh blood groups was performed using monoclonal antisera following classical hemagglutination test on a plate. Results A total of 5002 donors were collected during this COVID-19 pandemic period. Blood family/replacement donors represented 54.0% and new voluntary donors represented 52.6%. Comparison of HIV, HCV and syphilis markers seroprevalence showed no statistically significant difference between new voluntary donors and new family replacement donors (p>0.05). However, for HBV the seroprevalence was significantly higher in new family replacement donors (p=0,002). Conclusion The proper supply of blood was impacted by the COVID-19 pandemic meanwhile replacement donations had contributed to limiting the damage observed with blood shortages. However, the significant differences noted on the seroprevalences of transfusion-transmissible infections between voluntary non-paid donors and family/replacement donors strengthens WHO recommendations for the selection of volunteer non-paid donors to lower transfusion-transmissible HBV in sub Saharan Africa.","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"51 14","pages":""},"PeriodicalIF":3.2,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139126488","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}
Hemophagocytic lymphohistiocytosis (HLH), also defined as hemophagocytic syndrome (HPS), represents a potentially life-threatening hyperinflammatory syndrome, characterized by impaired function of cytotoxic T lymphocytes, natural killer cells and macrophages. The main clinical features of HLH are prolonged fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. Secondary HLH typically occurs in conjunction with severe infections, malignancies or autoimmune disorders and intensive chemotherapy, potentially complicating treatment of acute myeloid leukemia (AML) in around 10% of cases. Herein we report for the first time a case of HLH secondary to refractory/relapsed AML resolved after a second line treatment with azacitidine plus venetoclax, thus offering a new potential therapeutic perspective in the context of a life-threatening clinical scenario.
嗜血细胞淋巴组织细胞增多症（HLH）又称嗜血细胞综合征（HPS），是一种可能危及生命的高炎症综合征，其特点是细胞毒性 T 淋巴细胞、自然杀伤细胞和巨噬细胞的功能受损。HLH 的主要临床特征是长期发热、肝脾肿大、全血细胞减少、高甘油三酯血症、高铁蛋白血症以及骨髓、肝脏、脾脏或淋巴结的噬血细胞增多。继发性 HLH 通常与严重感染、恶性肿瘤或自身免疫性疾病以及强化化疗同时发生，约有 10% 的病例可能与急性髓性白血病（AML）的治疗并发。在此，我们首次报道了一例继发于难治性/复发性急性髓细胞白血病的HLH病例，该病例在接受阿扎胞苷联合venetoclax二线治疗后病情得到缓解，从而为危及生命的临床治疗提供了一个新的潜在治疗视角。
{"title":"Hemophagocytic lymphohistiocytosis secondary to refractory acute myeloid leukemia resolved after second line treatment with azacitidine plus venetoclax","authors":"Claudio Fozza","doi":"10.4084/mjhid.2024.011","DOIUrl":"https://doi.org/10.4084/mjhid.2024.011","url":null,"abstract":"Hemophagocytic lymphohistiocytosis (HLH), also defined as hemophagocytic syndrome (HPS), represents a potentially life-threatening hyperinflammatory syndrome, characterized by impaired function of cytotoxic T lymphocytes, natural killer cells and macrophages. The main clinical features of HLH are prolonged fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. Secondary HLH typically occurs in conjunction with severe infections, malignancies or autoimmune disorders and intensive chemotherapy, potentially complicating treatment of acute myeloid leukemia (AML) in around 10% of cases. Herein we report for the first time a case of HLH secondary to refractory/relapsed AML resolved after a second line treatment with azacitidine plus venetoclax, thus offering a new potential therapeutic perspective in the context of a life-threatening clinical scenario.","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"13 12","pages":""},"PeriodicalIF":3.2,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139126340","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":"Prof, Bruno Bizzi Obituary","authors":"Valerio De Stefano","doi":"10.4084/mjhid.2024.014","DOIUrl":"https://doi.org/10.4084/mjhid.2024.014","url":null,"abstract":"<jats:p>x</jats:p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":"51 32","pages":""},"PeriodicalIF":3.2,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139126792","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}
Ugo Testa, Simona Sica, E. Pelosi, G. Castelli, Giuseppe Leone
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.
{"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":"136 37","pages":""},"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}
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
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
{"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":"26 8","pages":""},"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}
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.
{"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":"25 2","pages":"0"},"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}
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