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The Features of COVID-19's Course and the Efficacy of the Gam-COVID-Vac Vaccine in Patients with Paroxysmal Nocturnal Hemoglobinuria. 新冠肺炎病程特点及γ-COVID-Vac疫苗对发作性夜间血红蛋白尿患者的疗效。
IF 0.9
Hematology Reports Pub Date : 2023-09-01 DOI: 10.3390/hematolrep15030052
Vadim Ptushkin, Evgeniya Arshanskaya, Olga Vinogradova, Dmitry Kudlay, Eugene Nikitin
{"title":"The Features of COVID-19's Course and the Efficacy of the Gam-COVID-Vac Vaccine in Patients with Paroxysmal Nocturnal Hemoglobinuria.","authors":"Vadim Ptushkin,&nbsp;Evgeniya Arshanskaya,&nbsp;Olga Vinogradova,&nbsp;Dmitry Kudlay,&nbsp;Eugene Nikitin","doi":"10.3390/hematolrep15030052","DOIUrl":"https://doi.org/10.3390/hematolrep15030052","url":null,"abstract":"<p><p>COVID-19 and other infectious diseases can exacerbate the course of paroxysmal nocturnal hemoglobinuria (PNH). The efficacy and safety of the Gam-COVID-Vac vaccine in patients with PNH has not been adequately studied. A retrospective, observational, cohort, non-comparative study was performed to assess the course of COVID-19 as well as the safety and efficacy of the Gam-COVID-Vac (Sputnik V) vaccine in patients with paroxysmal nocturnal hemoglobinuria (PNH). The study included data from 52 patients with PNH aged 18 to 75 years, 38 of whom received background therapy with eculizumab (Elizaria<sup>®</sup>) between March 2020 and January 2022. COVID-19 was diagnosed according to the results of PCR testing. The patients were divided into two groups for comparison of the incidence of COVID-19. Group 1 included non-vaccinated patients with PNH, and Group 2 included patients vaccinated prior to the onset of COVID-19. According to vaccination, patients were subdivided into non-vaccinated and vaccinated groups without signs of previous COVID-19 at the beginning of the analyzed period, and patients vaccinated half a year or more after recovery from COVID-19. Testing for anti-SARS-CoV-2 IgG levels was carried out in patients with PNH in the year after their COVID-19. Tests for anti-SARS-CoV-2 RBD IgG levels were performed on vaccinated patients. In total, 28 (53.8%) of the enrolled patients had COVID-19, including asymptomatic forms in 7 (25%) and mild forms in 16 (57%) patients. A total of 22 (42.3%) patients were fully vaccinated with Gam-COVID-Vac, of which 13 (25%) patients were vaccinated without the signs of previous SARS-CoV-2infection, and 9 (17.3%) patients were vaccinated after COVID-19. The number of patients who had COVID-19 was about two times higher in Group 1 (non-vaccinated; 24) (61.5%), whereas in Group 2 (vaccinated), the number of patients with COVID-19 was only 4 (30.8%). The proportion and number of patients who did not have COVID-19 was higher in the group of vaccinated patients (9; 69.2%) than in the group of non-vaccinated patients (15; 38.5%) (<i>p</i> = 0.054). In patients who had been infected with COVID-19, maximum concentrations of anti-SARS-CoV-2 IgG were observed 2-3 months after the acute infection phase, followed by a gradual decline by month 9-10. The mean <i>RBD</i> IgG concentration was higher in the group of patients who had been infected by COVID-19 than in the group of patients without COVID-19 (<i>p</i> = 0.047). Therapy type, including eculizumab, did not have a significant impact on RBD IgG titers (<i>p</i> > 0.05). Hospitalization was required in five (18%) patients, all of whom had breakthrough hemolysis and severe lung damage on CT scans. After the first dose, adverse events (AEs) were reported in 41% of the patients (body temperature increased in 18%; headache in 13.6%; and pain in joints in 4.5%; colitis exacerbation was observed in 4.5%). After the second dose, no AEs were reported. The performed study s","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10531158/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41120129","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
CD5+ Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type, Presenting as an Asymptomatic Nodule. CD5+原发性皮肤弥漫性大B细胞淋巴瘤,腿型,表现为无症状结节。
IF 0.9
Hematology Reports Pub Date : 2023-09-01 DOI: 10.3390/hematolrep15030053
Amy Xiao, Colleen J Beatty, Sonal Choudhary, Oleg E Akilov
{"title":"CD5+ Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type, Presenting as an Asymptomatic Nodule.","authors":"Amy Xiao,&nbsp;Colleen J Beatty,&nbsp;Sonal Choudhary,&nbsp;Oleg E Akilov","doi":"10.3390/hematolrep15030053","DOIUrl":"https://doi.org/10.3390/hematolrep15030053","url":null,"abstract":"<p><p>Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT), is a rare and aggressive variant of primary cutaneous lymphoma that typically expresses B cells as well as MUM1/IRF4, BCL2, and FOXP1, whereas BCL6 may be present or undetectable. We present a case of CD5+ PCDLBCL-LT presenting as a 6 mm pink-bluish nodule on the mid-left thigh, which was concerning for basal cell carcinoma. The histological examination reveals the presence of an intradermal proliferation of large, atypical CD5+, CD20+ BCL2+, BCL6+, MUM-1+, and Cyclin-D1+ lymphocytes in a nodular, diffuse interstitial and perivascular distribution. Because the patient presented with a small, single nodule, the systemic treatment of multiagent chemotherapy was avoided and localized electron beam radiation therapy with rituximab was initiated instead, achieving complete response. Early identification of PCDLBCL-LT is key for maximal therapeutic benefit and prognosis; it is important to consider PCDLBCL-LT on the differential when evaluating small, single nodules on the lower extremities of elderly patients.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10531099/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41111192","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Oral Manifestations: A Warning-Sign in Children with Hematological Disease Acute Lymphocytic Leukemia. 口腔表现:儿童血液病急性淋巴细胞白血病的警告信号。
IF 0.9
Hematology Reports Pub Date : 2023-08-24 DOI: 10.3390/hematolrep15030051
Sandra Clara Soares, Louis J D Roux, Ana Rita Castro, Cristina Cardoso Silva, Rita Rodrigues, Viviana M P Macho, Fátima Silva, Céu Costa
{"title":"Oral Manifestations: A Warning-Sign in Children with Hematological Disease Acute Lymphocytic Leukemia.","authors":"Sandra Clara Soares,&nbsp;Louis J D Roux,&nbsp;Ana Rita Castro,&nbsp;Cristina Cardoso Silva,&nbsp;Rita Rodrigues,&nbsp;Viviana M P Macho,&nbsp;Fátima Silva,&nbsp;Céu Costa","doi":"10.3390/hematolrep15030051","DOIUrl":"https://doi.org/10.3390/hematolrep15030051","url":null,"abstract":"<p><p>Acute lymphocytic leukemia (ALL) is the most frequent form of all childhood leukemias, mostly affecting children between 2 and 4 years old. Oral symptoms, such as mouth ulcers, mucositis, xerostomia, Herpes or Candidiasis, gingival enlargement and bleeding, petechiae, erythema, mucosal pallor and atrophic glossitis, are very common symptoms of ALL and can be early signs of the disease. Secondary and tertiary complications, a direct effect of chemo and radiotherapy, are associated with more severe bleeding, higher susceptibility to infections, ulcerations, inflammation of the mucous membranes, osteoradionecrosis, xerostomia, taste alterations, trismus, carious lesions and dental abnormalities. Immunotherapy, though less toxic, causes oral dysesthesia and pain. Overall, the effects in the oral cavity are transient but there are long-term consequences like caries, periodontal disease and tooth loss that impair endodontic and orthodontic treatments. Also, dental abnormalities resulting from disturbed odontogenesis are known to affect a child's quality of life. The medical dentist should identify these complications and perform appropriate oral care in tandem with other health professionals. Thus, poor oral hygiene can lead to systemic ALL complications. The aim of this review is to describe the oral complications in children with ALL who are undergoing chemo, radio or immunotherapy.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10530953/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41178624","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Concomitant Presence of Hb Agrinio and - -Med Deletion in a Greek Male Patient with Hemoglobinopathy H: More Severe Phenotype and Literature Review. 一名希腊男性H型血红蛋白病患者同时存在Hb Agrinio和- med缺失:更严重的表型和文献综述。
IF 0.9
Hematology Reports Pub Date : 2023-08-08 DOI: 10.3390/hematolrep15030050
Michael D Diamantidis, Stefania Pitsava, Omar Zayed, Ioanna Argyrakouli, Konstantinos Karapiperis, Christos Chatzoulis, Evangelos Alexiou, Achilles Manafas, Evangelos Tsangalas, Konstantinos Karakoussis
{"title":"Concomitant Presence of Hb Agrinio and - -Med Deletion in a Greek Male Patient with Hemoglobinopathy H: More Severe Phenotype and Literature Review.","authors":"Michael D Diamantidis,&nbsp;Stefania Pitsava,&nbsp;Omar Zayed,&nbsp;Ioanna Argyrakouli,&nbsp;Konstantinos Karapiperis,&nbsp;Christos Chatzoulis,&nbsp;Evangelos Alexiou,&nbsp;Achilles Manafas,&nbsp;Evangelos Tsangalas,&nbsp;Konstantinos Karakoussis","doi":"10.3390/hematolrep15030050","DOIUrl":"https://doi.org/10.3390/hematolrep15030050","url":null,"abstract":"<p><p>Hemoglobin (Hb) Agrinio is a rare non-deletional a-globin mutation observed almost exclusively in Greek, Spanish or other Mediterranean families. The clinical manifestations of a carrier of a single Hb Agrinio mutation (single heterozygosity) depend on the concomitant presence or absence of other mutations or variants in the beta, alpha or other modifying genes. We present a Greek patient harboring a Hb Agrinio variant plus the - -Med alpha deletional allele, having an infrequent severe form of alpha thalassemia, in contrast to the typical alpha thalassemic patient and requiring regular red blood cell (RBC) transfusions and chelation treatment. We also provide a concise literature review regarding alpha thalassemic hemoglobin variants and their molecular and clinical combinations. A phase 2, double-blind, randomized, placebo-controlled, multicenter clinical trial to determine the efficacy and safety of luspatercept (BMS-986346/ACE-536) for the treatment of anemia in adults with alpha thalassemia with the participation of our center is currently recruiting patients (NCT05664737).</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10443284/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10060894","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Impact of Quantitative Computed Tomography-Based Analysis of Abdominal Adipose Tissue in Patients with Lymphoma. 基于定量计算机断层扫描分析腹部脂肪组织对淋巴瘤患者的影响。
IF 0.9
Hematology Reports Pub Date : 2023-08-04 DOI: 10.3390/hematolrep15030049
Federico Greco, Bruno Beomonte Zobel, Carlo Augusto Mallio
{"title":"Impact of Quantitative Computed Tomography-Based Analysis of Abdominal Adipose Tissue in Patients with Lymphoma.","authors":"Federico Greco,&nbsp;Bruno Beomonte Zobel,&nbsp;Carlo Augusto Mallio","doi":"10.3390/hematolrep15030049","DOIUrl":"https://doi.org/10.3390/hematolrep15030049","url":null,"abstract":"<p><p>Quantitative abdominal adipose tissue analysis is important for obtaining information about prognosis and clinical outcomes on a wide array of diseases. In recent years, the effects of abdominal adipose tissue compartments in patients with lymphoma and the changes in their distribution after therapies have been studied. This information could facilitate the improvement of therapies in patients with lymphoma, to prevent or treat both visceral obesity and sarcopenia. Opportunistic analysis of body composition on computed tomography (CT) images might contribute to the improvement of patient management and clinical outcomes together with implementation of targeted patient-tailored therapies. The purpose of this literature review is to describe the role of CT to evaluate abdominal adipose tissue quantity and distribution in patients with lymphoma.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-08-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10443254/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10058806","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Efficacy of Bendamustine, Pomalidomide, and Dexamethasone (BPD) Regimen in Relapsed/Refractory Extramedullary Myeloma: A Retrospective Single-Centre Study, Real-Life Experience. 苯达莫司汀、波马度胺和地塞米松(BPD)方案治疗复发/难治性髓外骨髓瘤的疗效:一项回顾性单中心研究,现实经验
IF 0.9
Hematology Reports Pub Date : 2023-08-02 DOI: 10.3390/hematolrep15030048
İbrahim Halil Açar, Birol Güvenç
{"title":"Efficacy of Bendamustine, Pomalidomide, and Dexamethasone (BPD) Regimen in Relapsed/Refractory Extramedullary Myeloma: A Retrospective Single-Centre Study, Real-Life Experience.","authors":"İbrahim Halil Açar,&nbsp;Birol Güvenç","doi":"10.3390/hematolrep15030048","DOIUrl":"https://doi.org/10.3390/hematolrep15030048","url":null,"abstract":"<p><strong>Background and objectives: </strong>Relapsed/refractory extramedullary myeloma (RREMM) is an uncommon and aggressive subtype of multiple myeloma defined by plasma cell proliferation outside the bone marrow. Therapeutic options for RREMM are limited, and the prognosis is generally unfavorable. This research aimed to assess the effectiveness of the bendamustine, pomalidomide, and dexamethasone (BPD) regimen in patients with RREMM.</p><p><strong>Material and methods: </strong>We carried out a retrospective investigation of 11 RREMM patients who underwent BPD treatment. The primary endpoint was progression-free survival. The secondary endpoints of the study were two-year survival and overall response rate (ORR). We analyzed the sociodemographic and clinical features of the patients.</p><p><strong>Results: </strong>The average age of the patients was 62 years. They had a median of four prior treatment lines, and eight patients had previously received autologous stem-cell transplantation. After eight BPD treatment cycles, the ORR stood at 54%, with one very good partial response (VGPR), five partial responses (PR), three progressive diseases (PD), and two stable diseases (SD). The median follow-up was 15 months, with a two-year PFS rate of 71.3% and a two-year survival rate of 81.8%.</p><p><strong>Conclusions: </strong>The BPD regimen demonstrated promising effectiveness in RREMM patients, yielding favorable ORR and survival rates. To corroborate these findings and explore additional treatment alternatives for this patient group, larger prospective studies are required.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10443238/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10060889","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
How I Manage Chronic Lymphocytic Leukemia. 我是如何管理慢性淋巴细胞白血病的?
IF 1.1
Hematology Reports Pub Date : 2023-08-01 DOI: 10.3390/hematolrep15030047
Patrice Nasnas, Claudio Cerchione, Gerardo Musuraca, Giovanni Martinelli, Alessandra Ferrajoli
{"title":"How I Manage Chronic Lymphocytic Leukemia.","authors":"Patrice Nasnas, Claudio Cerchione, Gerardo Musuraca, Giovanni Martinelli, Alessandra Ferrajoli","doi":"10.3390/hematolrep15030047","DOIUrl":"10.3390/hematolrep15030047","url":null,"abstract":"<p><p>Chronic lymphocytic leukemia (CLL), is a hematologic malignancy characterized by the uncontrolled proliferation of mature B lymphocytes. CLL is the most prevalent leukemia in Western countries. Its presentation can range from asymptomatic with the incidental finding of absolute lymphocytosis on a routine blood test, to symptomatic disease requiring immediate intervention. Prognosis of the disease is defined by the presence or absence of specific mutations such as TP53, chromosomal abnormalities such as del(17p), a type of IGHV mutational status, and elevation of B2M and LDH. Treatment of CLL in the United States and Europe has evolved over the recent years thanks to the development of targeted therapies. The standard of care has shifted from traditional chemoimmunotherapy approaches to targeted therapies including Bruton tyrosine kinase inhibitors (BTKis) and BCL2 inhibitors, administered either as monotherapy or in combination with CD20 monoclonal antibodies. Several clinical trials have also recently evaluated combinations of BTKi and venetoclax and showed the combination to be well tolerated and able to induce deep remissions. Targeted therapies have a good safety profile overall; however, they also have unique toxicities that are important to recognize. Diarrhea, fatigue, arthralgia, infections, cytopenias, bleeding, and cardiovascular toxicities (including atrial fibrillation, ventricular arrhythmias, and hypertension) are the adverse events (AEs) commonly associated with BTKis. Initiation of therapy with venetoclax requires close monitoring because of the risk for tumor lysis syndrome associated with this agent, particularly in patients with a high disease burden. Development of newer target therapies is ongoing and the therapeutic landscape in CLL is expanding rapidly.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10443285/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10058807","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Safe and Effective Administration of Caplacizumab in COVID-19-Associated Thrombotic Thrombocytopenic Purpura. Caplacizumab在covid -19相关血栓性血小板减少性紫癜中的安全有效应用
IF 0.9
Hematology Reports Pub Date : 2023-07-20 DOI: 10.3390/hematolrep15030046
Antonella Bruzzese, Ernesto Vigna, Dario Terzi, Sonia Greco, Enrica Antonia Martino, Valeria Vangeli, Francesco Mendicino, Eugenio Lucia, Virginia Olivito, Caterina Labanca, Rosellina Morelli, Antonino Neri, Fortunato Morabito, Francesco Zinno, Antonio Mastroianni, Massimo Gentile
{"title":"Safe and Effective Administration of Caplacizumab in COVID-19-Associated Thrombotic Thrombocytopenic Purpura.","authors":"Antonella Bruzzese,&nbsp;Ernesto Vigna,&nbsp;Dario Terzi,&nbsp;Sonia Greco,&nbsp;Enrica Antonia Martino,&nbsp;Valeria Vangeli,&nbsp;Francesco Mendicino,&nbsp;Eugenio Lucia,&nbsp;Virginia Olivito,&nbsp;Caterina Labanca,&nbsp;Rosellina Morelli,&nbsp;Antonino Neri,&nbsp;Fortunato Morabito,&nbsp;Francesco Zinno,&nbsp;Antonio Mastroianni,&nbsp;Massimo Gentile","doi":"10.3390/hematolrep15030046","DOIUrl":"https://doi.org/10.3390/hematolrep15030046","url":null,"abstract":"<p><p>Thrombotic thrombocytopenic purpura (TTP) is a potentially life-threatening, rare acute thrombotic microangiopathy (TMA), caused by a severe ADAMTS13 deficiency. As the COVID-19 pandemic rapidly spread around the globe, much data about the pathogenicity of this virus were published. Soon after the detection of the first cases of COVID-19, it was clear that there was a wide range of COVID coagulopathy manifestations, such as deep venous thrombosis, pulmonary thromboembolism, and thrombotic microangiopathies. In the literature, little data have been reported about the association between TTP and COVID-19, and the treatment of COVID-19-associated TTP is still under debate. Here we present the case of a 46-year-old woman who developed a COVID-associated TTP, successfully treated with plasma exchange (PEX), steroids, and caplacizumab.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10366750/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9874664","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Acute Promyelocytic Leukemia and Brugada Syndrome: A Report on the Safety of Arsenic Trioxide/All-Trans-Retinoic Acid Therapy. 急性早幼粒细胞白血病和Brugada综合征:三氧化二砷/全反式视黄酸治疗的安全性报告。
IF 0.9
Hematology Reports Pub Date : 2023-07-17 DOI: 10.3390/hematolrep15030045
Giorgio Rosati, Sofia Camerlo, Matteo Dalmazzo, Melissa Padrini, Tiziano Tommaso Busana, Marco De Gobbi, Alessandro Fornari, Alessandro Morotti
{"title":"Acute Promyelocytic Leukemia and Brugada Syndrome: A Report on the Safety of Arsenic Trioxide/All-Trans-Retinoic Acid Therapy.","authors":"Giorgio Rosati,&nbsp;Sofia Camerlo,&nbsp;Matteo Dalmazzo,&nbsp;Melissa Padrini,&nbsp;Tiziano Tommaso Busana,&nbsp;Marco De Gobbi,&nbsp;Alessandro Fornari,&nbsp;Alessandro Morotti","doi":"10.3390/hematolrep15030045","DOIUrl":"https://doi.org/10.3390/hematolrep15030045","url":null,"abstract":"<p><p>Acute promyelocytic leukemia (APL) is a rare and aggressive form of acute myeloid leukemia (AML). Instead of cytotoxic chemotherapy, a combination of all-trans-retinoic acid (ATRA) and arsenic trioxide (ATO) represents front-line therapy in low-risk patients. However, the therapeutic approach could be challenging in the case of a concomitant diagnosis of Brugada syndrome (BrS), a genetic disease characterized by an increased risk of arrhythmias and sudden cardiac death. Here, we present the case of a BrS patient who has been diagnosed with low-risk APL and treated with ATRA and ATO without observing arrhythmic events. In particular, we highlight the difficulties encountered by clinicians during the diagnostic work-up and the choice of the best treatment for these patients.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10366891/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9872234","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Effects of Sirolimus Treatment on Fetal Hemoglobin Production and Response to SARS-CoV-2 Vaccination: A Case Report Study. 西罗莫司治疗对胎儿血红蛋白生成和对SARS-CoV-2疫苗应答的影响:一项病例报告研究
IF 0.9
Hematology Reports Pub Date : 2023-07-12 DOI: 10.3390/hematolrep15030044
Maria Rita Gamberini, Cristina Zuccato, Matteo Zurlo, Lucia Carmela Cosenza, Alessia Finotti, Roberto Gambari
{"title":"Effects of Sirolimus Treatment on Fetal Hemoglobin Production and Response to SARS-CoV-2 Vaccination: A Case Report Study.","authors":"Maria Rita Gamberini,&nbsp;Cristina Zuccato,&nbsp;Matteo Zurlo,&nbsp;Lucia Carmela Cosenza,&nbsp;Alessia Finotti,&nbsp;Roberto Gambari","doi":"10.3390/hematolrep15030044","DOIUrl":"https://doi.org/10.3390/hematolrep15030044","url":null,"abstract":"<p><p>The β-thalassemias are a group of monogenic hereditary hematological disorders caused by deletions and/or mutations of the β-globin gene, leading to low or absent production of adult hemoglobin (HbA). For β-thalassemia, sirolimus has been under clinical consideration in two trials (NCT03877809 and NCT04247750). A reduced immune response to anti-SARS-CoV-2 vaccination has been reported in organ recipient patients treated with the immunosuppressant sirolimus. Therefore, there was some concern regarding the fact that monotherapy with sirolimus would reduce the antibody response after SARS-CoV-2 vaccination. In the representative clinical case reported in this study, sirolimus treatment induced the expected increase of fetal hemoglobin (HbF) but did not prevent the production of anti-SARS-CoV-2 IgG after vaccination with mRNA-1273 (Moderna). In our opinion, this case report should stimulate further studies on β-thalassemia patients under sirolimus monotherapy in order to confirm the safety (or even the positive effects) of sirolimus with respect to the humoral response to anti-SARS-CoV-2 vaccination. In addition, considering the extensive use of sirolimus for the treatment of other human pathologies (for instance, in organ transplantation, systemic lupus erythematosus, autoimmune cytopenia, and lymphangioleiomyomatosis), this case report study might be of general interest, as large numbers of patients are currently under sirolimus treatment.</p>","PeriodicalId":12829,"journal":{"name":"Hematology Reports","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2023-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10366771/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9865516","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","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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