Journal of hematology最新文献

{"title":"Local and Systemic Immunity During Five Vaccinations Against SARS-CoV-2 in Zanubrutinib-Treated Patients With Chronic Lymphocytic Leukemia.","authors":"Maria Andersson,&nbsp;Jinghua Wu,&nbsp;David Wullimann,&nbsp;Yu Gao,&nbsp;Mikael Aberg,&nbsp;Sandra Muschiol,&nbsp;Katie Healy,&nbsp;Sabrina Naud,&nbsp;Gordana Bogdanovic,&nbsp;Marzia Palma,&nbsp;Hakan Mellstedt,&nbsp;Puran Chen,&nbsp;Hans-Gustaf Ljunggren,&nbsp;Lotta Hansson,&nbsp;Margaret Sallberg Chen,&nbsp;Marcus Buggert,&nbsp;Hanna M Ingelman-Sundberg,&nbsp;Anders Osterborg","doi":"10.14740/jh1140","DOIUrl":"https://doi.org/10.14740/jh1140","url":null,"abstract":"<p><strong>Background: </strong>Patients with chronic lymphocytic leukemia (CLL) are vulnerable to coronavirus disease 2019 (COVID-19) and are at risk of inferior response to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination, especially if treated with the first-generation Bruton's tyrosine kinase inhibitor (BTKi) ibrutinib. We aimed to evaluate the impact of the third-generation BTKi, zanubrutinib, on systemic and mucosal response to SARS-CoV-2 vaccination.</p><p><strong>Methods: </strong>Nine patients with CLL with ongoing zanubrutinib therapy were included and donated blood and saliva during SARS-CoV-2 vaccination, before vaccine doses 3 and 5 and 2 - 3 weeks after doses 3, 4, and 5. Ibrutinib-treated control patients (n = 7) and healthy aged-matched controls (n = 7) gave blood 2 - 3 weeks after vaccine dose 5. We quantified reactivity and neutralization capacity of SARS-CoV-2-specific IgG and IgA antibodies (Abs) in both serum and saliva, and reactivity of T cells activated with viral peptides.</p><p><strong>Results: </strong>Both zanubrutinib- and ibrutinib-treated patients had significantly, up to 1,000-fold, lower total spike-specific Ab levels after dose 5 compared to healthy controls (P < 0.01). Spike-IgG levels in serum from zanubrutinib-treated patients correlated well to neutralization capacity (r = 0.68; P < 0.0001) and were thus functional. Mucosal immunity (specific IgA in serum and saliva) was practically absent in zanubrutinib-treated patients even after five vaccine doses, whereas healthy controls had significantly higher levels (tested in serum after vaccine dose 5) (P < 0.05). In contrast, T-cell reactivity against SARS-CoV-2 peptides was equally high in zanubrutinib- and ibrutinib-treated patients as in healthy control donors.</p><p><strong>Conclusions: </strong>In our small cohort of zanubrutinib-treated CLL patients, we conclude that up to five doses of SARS-CoV-2 vaccination induced no detectable IgA mucosal immunity, which likely will impair the primary barrier defence against the infection. Systemic IgG responses were also impaired, whereas T-cell responses were normal. Further and larger studies are needed to evaluate the impact of these findings on disease protection.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 4","pages":"170-175"},"PeriodicalIF":1.2,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/3f/36/jh-12-170.PMC10482612.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10570754","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}

{"title":"Harnessing the Immune System: An Effective Way to Manage Diffuse Large B-Cell Lymphoma.","authors":"Nathan Visweshwar, Juan Felipe Rico, Robert Killeen, Arumugam Manoharan","doi":"10.14740/jh1112","DOIUrl":"10.14740/jh1112","url":null,"abstract":"<p><p>Diffuse large B-cell lymphoma (DLBCL) is a heterogenous hematological disorder with malignant potential controlled by immunological characteristics of the tumor microenvironment. Rapid breakthrough in the molecular pathways has made immunological approaches the main anchor in the management of DLBCL, with or without chemotherapeutic agents. Rituximab was the first monoclonal antibody approved for the treatment of DLBCL. Following rituximab that transformed the therapeutic landscape, other novel immunological agents including chimeric antigen T-cell therapy have reshaped the management of relapsed/refractory DLBCL. However, resistance and refractory state remain a challenge in the management of DLBCL. For this literature review, we screened articles from Medline, Embase, Cochrane databases and the European/North American guidelines from March 2010 through October 2022 for DLBCL. Here we discuss immunological agents that will significantly affect future treatment of this aggressive type of lymphoma.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 4","pages":"145-160"},"PeriodicalIF":1.3,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/35/91/jh-12-145.PMC10482611.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10570755","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}

{"title":"Reduced-Intensity Anthracycline-Free Chemoimmunotherapy in Elderly Patients With Newly Diagnosed or Relapsed Diffuse Large B-Cell Lymphoma.","authors":"Binoy Yohannan,&nbsp;Adan Rios","doi":"10.14740/jh1144","DOIUrl":"https://doi.org/10.14740/jh1144","url":null,"abstract":"<p><p>Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL); it has a cure rate of approximately 50% with standard anthracycline-based chemoimmunotherapy. However, the clinical outcomes of elderly unfit/frail DLBCL patients remain suboptimal due to poor tolerance of anthracycline-containing regimens. Herein, we report a series of seven elderly unfit patients with DLBCL who were treated with a reduced-intensity anthracycline-free chemoimmunotherapy (rituximab, cyclophosphamide, vincristine, and prednisone) regimen combined with lenalidomide (R₂-COP). Five patients received R₂-COP as first-line therapy, and two patients were treated for relapsed DLBCL. Four patients with newly diagnosed DLBCL and two with relapsed disease achieved complete remission. The R₂-COP regimen was well tolerated. Interim positron emission tomography (PET) scans in four patients after two to three cycles showed a complete metabolic response. At a median follow-up of 24 months, six patients remain in complete remission. R₂-COP is an effective anthracycline-free regimen with encouraging clinical activity in elderly DLBCL patients who are unfit for standard anthracycline-containing regimens.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 4","pages":"176-186"},"PeriodicalIF":1.2,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/67/f7/jh-12-176.PMC10482608.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10570757","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}

{"title":"Could the Crosstalk Between Myeloid-Derived-Suppressor Cells and Regulatory T Cells Have a Role in Beta-Thalassemia?","authors":"Asmaa M Zahran,&nbsp;Omnia El-Badawy,&nbsp;Eman R Badawy,&nbsp;Khalid I Elsayh,&nbsp;Eman F Gad,&nbsp;Khaled Saad,&nbsp;Khalid Hashim Mahmoud,&nbsp;Amira Elhoufey,&nbsp;Hamad Ghaleb Dailah,&nbsp;Marwa Ghazaly","doi":"10.14740/jh1149","DOIUrl":"https://doi.org/10.14740/jh1149","url":null,"abstract":"<p><strong>Background: </strong>Secondary iron overload, alloimmunization, and increased risk of infection are common complications in patients with transfusion-dependent thalassemia (TDT). Regulatory T cells (Tregs) and myeloid-derived suppressor cells (MDSCs) play an essential role in preventing excessive immune response. This research aimed to study the interaction between Tregs and MDSCs in TDT patients and to evaluate the association of these cell types with disease severity.</p><p><strong>Methods: </strong>This case-control study included 26 patients with TDT and 23 healthy, age- and sex-matched controls. All patients were investigated for complete blood count (CBC), serum ferritin, and flow cytometric analysis of peripheral blood to detect Tregs, MDSCs, and MDSC subsets.</p><p><strong>Results: </strong>A significant increase was observed in the frequencies of Tregs and MDSCs, particularly monocytic MDSCs (MO-MDSCs), in TDT patients compared with controls. The frequencies of these cells showed a direct association with ferritin level and total leukocyte count and an inverse association with hemoglobin level. Furthermore, a positive correlation was observed between Tregs and each of the total MDSCs and MO-MDSCs.</p><p><strong>Conclusions: </strong>Levels of Tregs and MDSCs increased in TDT and may probably have a role in suppressing the active immune systems of TDT patients.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 4","pages":"161-169"},"PeriodicalIF":1.2,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/02/1e/jh-12-161.PMC10482610.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10276536","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}

{"title":"An Aggressive Course of Transformed Splenic Diffuse Red Pulp Small B-Cell Lymphoma With Novel Somatic Loss-of-Function Mutation in <i>RB1</i>.","authors":"Lisa-Maj Christensen,&nbsp;Marianne Tang Severinsen,&nbsp;Pragya Katoch,&nbsp;Andreas Kiesbye Ovlisen,&nbsp;Thor Hoyer,&nbsp;Paw Jensen,&nbsp;Karen Dybkaer,&nbsp;Daniel Tuyet Kristensen","doi":"10.14740/jh1132","DOIUrl":"https://doi.org/10.14740/jh1132","url":null,"abstract":"<p><p>Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is an extremely rare B-cell lymphoma. The disease is typically indolent and treatment with splenectomy usually results in durable remissions. Here, we present a case of an extremely aggressive course of SDRPL with transformation to diffuse large B-cell lymphoma and multiple relapses immediately following cessation of immunochemotherapy. We provide results from whole-exome sequencing from debut of SDRPL and from following transformed stages and identified a novel somatic mutation in <i>RB1</i> as the possible driver of this aggressive disease, which has not been reported earlier in SDRPL.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 3","pages":"118-122"},"PeriodicalIF":1.2,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/7b/a2/jh-12-118.PMC10332862.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9816293","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}

{"title":"Hyperammonemic Encephalopathy: A Rare Presentation of Relapsed Multiple Myeloma.","authors":"Sasmith R Menakuru,&nbsp;Mona Atta,&nbsp;Nischala Ammannagari,&nbsp;Mohamad Younes","doi":"10.14740/jh1097","DOIUrl":"https://doi.org/10.14740/jh1097","url":null,"abstract":"<p><p>Hyperammonemia is a rare cause of encephalopathy in multiple myeloma in the absence of hepatic involvement. This is the only reported case of a 74-year-old man who presented with multiple myeloma and achieved complete remission but developed hyperammonemia afterward. He was aggressively treated with a combination of chemotherapy and immunotherapy, with a resolution of his encephalopathy; however, within one month, he relapsed with encephalopathy. He ultimately decided to pursue comfort-care measures. The authors conclude that hyperammonemia in multiple myeloma is a rare but important differential in patients with encephalopathy of unknown causes. Aggressive treatment is of the utmost importance due to the high mortality associated with the condition.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 3","pages":"128-132"},"PeriodicalIF":1.2,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f6/75/jh-12-128.PMC10332859.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9816296","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}

{"title":"Amplification of Chromosome 1q Predicts Poor Overall Survival in Newly Diagnosed Multiple Myeloma Patients.","authors":"Matevz Skerget,&nbsp;Barbara Skopec,&nbsp;Samo Zver,&nbsp;Helena Podgornik","doi":"10.14740/jh1137","DOIUrl":"https://doi.org/10.14740/jh1137","url":null,"abstract":"<p><strong>Background: </strong>Chromosome 1q copy number alterations are common in newly diagnosed patients with multiple myeloma, and in most published studies, there is no distinction made between three copies or the addition of at least four copies. The impact of these copy number alterations on patient outcome and optimal treatment is not fully understood.</p><p><strong>Methods: </strong>We retrospectively analyzed 136 transplant eligible patients with newly diagnosed multiple myeloma from our national registry, who were treated with first autologous stem cell transplantation (aHSCT) between January 1, 2018, and December 31, 2021. The primary endpoint was overall survival.</p><p><strong>Results: </strong>Patients with at least four copies of chromosome 1q had the poorest prognosis, with an overall survival of only 28.3 months. In multivariate analysis, four copies of chromosome 1q were the only statistically significant factor for overall survival.</p><p><strong>Conclusions: </strong>Despite the use of novel agents, transplantation, and maintenance therapy, patients with a gain of four copies of chromosome 1q have a very poor survival rate. Therefore, prospective studies using immunotherapy in this patient population are necessary.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 3","pages":"109-113"},"PeriodicalIF":1.2,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/09/e8/jh-12-109.PMC10332865.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9818443","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}

{"title":"Real-World Data of Crizanlizumab in Sickle Cell Disease: A Single-Center Analysis.","authors":"Halle Cheplowitz,&nbsp;Shanna Block,&nbsp;Jessica Groesbeck,&nbsp;Stefanie Sacknoff,&nbsp;Anthony L Nguyen,&nbsp;Srila Gopal","doi":"10.14740/jh1127","DOIUrl":"https://doi.org/10.14740/jh1127","url":null,"abstract":"<p><strong>Background: </strong>Crizanlizumab was approved by the United States Food and Drug Administration agency in 2019 for decreasing vaso-occlusive events (VOEs) in sickle cell disease (SCD). Data regarding the use of crizanlizumab in the real-world setting are limited. Our goal was to identify patterns of crizanlizumab prescriptions in our SCD program and evaluate the benefits and identify barriers to its use in our SCD clinic.</p><p><strong>Methods: </strong>We conducted a retrospective analysis of patients who received crizanlizumab at our institution between July 2020 and January 2022. We compared acute care usage patterns before and after initiation of crizanlizumab, adherence to treatment, discontinuation and reasons for discontinuation. High utilizers of hospital-based services were defined as those with more than one visit to the emergency department (ED) per month or more than three visits to the day infusion program per month.</p><p><strong>Results: </strong>Fifteen patients received at least one dose of crizanlizumab 5 mg/kg of actual body weight during the study period. The average number of acute care visits decreased following crizanlizumab initiation but was not statistically significant (20 visits vs. 10 visits, P = 0.07). Among high users of hospital-based services, the average number of acute care visits decreased after initiation of crizanlizumab (40 vs. 16, P = 0.005). Only five patients included in this study remained on crizanlizumab 6 months after initiation.</p><p><strong>Conclusion: </strong>Our study suggests that crizanlizumab use may be helpful in decreasing acute care visits in SCD, particularly among high utilizers of hospital-based acute care services. However, the discontinuation rate in our cohort was extremely high, and further evaluation of efficacy and causes contributing to discontinuation in larger cohorts is warranted.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 3","pages":"105-108"},"PeriodicalIF":1.2,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/d3/19/jh-12-105.PMC10332863.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9818446","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}

{"title":"Importance of the Third Trimester Complete Blood Count: A Case Report on Aplastic Anemia in Pregnancy.","authors":"Jaclyn Del Pozzo,&nbsp;Insaf Kouba,&nbsp;Theodore Goldman,&nbsp;Jolene Muscat","doi":"10.14740/jh1131","DOIUrl":"https://doi.org/10.14740/jh1131","url":null,"abstract":"<p><p>Aplastic anemia (AA) poses a significant threat to maternal and fetal health throughout the perinatal period. Diagnosis is based on complete blood count (CBC) and bone marrow biopsy with treatment varying based on severity of disease. This report highlights a case of AA incidentally identified by the third trimester CBC drawn in the outpatient office. Patient was referred for inpatient management to mobilize a multidisciplinary team of healthcare professionals including obstetricians, hematologists, and anesthesiologists to optimize maternal and fetal outcome. The patient received blood and platelet transfusions prior to delivering a healthy liveborn infant by cesarean section. This case highlights the importance for routine third trimester CBC screening to identify potential complications and decrease maternal and fetal morbidity and mortality.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 3","pages":"114-117"},"PeriodicalIF":1.2,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/11/96/jh-12-114.PMC10332860.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9816292","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}

{"title":"Sickle Cell Trait: Is It Always Benign?","authors":"Tyiesha Sharron Brown,&nbsp;Rachaita Lakra,&nbsp;Samip Master,&nbsp;Poornima Ramadas","doi":"10.14740/jh958","DOIUrl":"https://doi.org/10.14740/jh958","url":null,"abstract":"<p><p>Sickle cell disease is a well-known homozygous inherited hemoglobinopathy that causes vaso-occlusive phenomena and chronic hemolysis. Vaso-occlusion results in sickle cell crisis and can eventually lead to complications involving multiple organ systems. However, the heterozygous counterpart, sickle cell trait (SCT) has less clinical significance as these patients are generally asymptomatic. This case series examines three unrelated patients with SCT ranging from the age of 27 to 61 years, who presented with pain in multiple long bones. Hemoglobin electrophoresis confirmed a diagnosis of SCT. Radiographic images of the affected sites showed osteonecrosis (ON). Interventions included pain management and bilateral hip replacement in two of the patients. Historically, vaso-occlusive disease in patients with SCT with no evidence of hemolysis or other hallmark findings of sickle cell disease is rare. There are limited reported cases of ON in SCT patients. Clinicians should explore other hemoglobinopathies not tested on routine hemoglobin electrophoresis and alternative risk factors for ON in these patients.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"12 3","pages":"123-127"},"PeriodicalIF":1.2,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/19/45/jh-12-123.PMC10332861.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9816295","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}