Journal Description
Hematology Reports
Hematology Reports
- formerly Hematology Reviews - is an international, peer-reviewed, open access journal on all aspects of prevention, diagnosis and management of disorders of the blood, published quarterly online by MDPI (from Volume 14, Issue 1 - 2022). The Society of Hematologic Oncology Italy (SOHO ITALY) is affiliated with Hematology Reports and their members receive discounts on the article processing charges.
- Open Access— free for readers, with article processing charges (APC) paid by authors or their institutions.
- High Visibility: indexed within Scopus, ESCI (Web of Science), PMC, PubMed, Embase, and other databases.
- Rapid Publication: manuscripts are peer-reviewed and a first decision is provided to authors approximately 38.9 days after submission; acceptance to publication is undertaken in 6.8 days (median values for papers published in this journal in the second half of 2023).
- Recognition of Reviewers: APC discount vouchers, optional signed peer review, and reviewer names published annually in the journal.
Impact Factor:
0.9 (2022);
5-Year Impact Factor:
1.0 (2022)
Latest Articles
Emerging Role of [18F]FLT PET/CT in Lymphoid Malignancies: A Review of Clinical Results
Hematol. Rep. 2024, 16(1), 32-41; https://doi.org/10.3390/hematolrep16010004 - 11 Jan 2024
Abstract
Fluorine-18 fluorodeoxyglucose ([18F]FDG) is nowadays the leading positron emission tomography (PET) tracer for routine clinical work-ups in hematological malignancies; however, it is limited by false positive findings. Notably, false positives can occur in inflammatory and infective cases or in necrotic tumors
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Fluorine-18 fluorodeoxyglucose ([18F]FDG) is nowadays the leading positron emission tomography (PET) tracer for routine clinical work-ups in hematological malignancies; however, it is limited by false positive findings. Notably, false positives can occur in inflammatory and infective cases or in necrotic tumors that are infiltrated by macrophages and other inflammatory cells. In this context, 3′-deoxy-3′-[18F]fluorothymidine ([18F]FLT) has been shown to be a promising imaging biomarker of hematological malignant cell proliferation. In this review, a total of 15 papers were reviewed to collect literature data regarding the clinical application of [18F]FLT PET/CT in hematological malignancies. This imaging modality seems to be a suitable tool for noninvasive assessment of tumor grading, also showing a correlation with Ki-67 immunostaining. Moreover, [18F]FLT PET/CT demonstrated high sensitivity in detecting aggressive lymphoma lesions, especially when applying a standardized uptake value (SUV) cutoff of 3. At baseline, the potential of [18F]FLT imaging as a predictive tool is demonstrated by the low tracer uptake in patients with a complete response. However, its use is limited in evaluating bone diseases due to its high physiological uptake in bone marrow. Interim [18F]FLT PET/CT (iFLT) has the potential to identify high-risk patients with greater precision than [18F]FDG PET/CT, optimizing risk-adapted therapy strategies. Moreover, [18F]FLT uptake showed a greater ability to differentiate tumor from inflammation compared to [18F]FDG, allowing the reduction of false-positive findings and making the first one a more selective tracer. Finally, FLT emerges as a superior independent predictor of PFS and OS compared to FDG and ensures a reliable early response assessment with greater accuracy and predictive value.
Full article
(This article belongs to the Special Issue Comparison between 18F-FDG and Alternative Tracers for the Assessment of Hematological Ma-Lignancies)
Open AccessCase Report
Plasmacytoma in the Maxillary Jaw: A Diagnostic and Therapeutic Challenge
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Hematol. Rep. 2024, 16(1), 22-31; https://doi.org/10.3390/hematolrep16010003 - 04 Jan 2024
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Plasmacytoma is a neoplastic disorder originating from plasma cells, with bone and soft tissue being common sites of manifestation. This report presents the clinical and radiological findings of a 65-year-old female patient who presented with an exophytic lesion in the upper right lateral
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Plasmacytoma is a neoplastic disorder originating from plasma cells, with bone and soft tissue being common sites of manifestation. This report presents the clinical and radiological findings of a 65-year-old female patient who presented with an exophytic lesion in the upper right lateral incisor region. The lesion appeared as a unilocular radiotransparent area in imaging tests. Following an excisional biopsy, histological and immunohistochemical evaluations confirmed the presence of mature plasmacellular elements and small infiltrates of B and T lymphocytes. The patient did not exhibit systemic manifestations of multiple myeloma. Surgical intervention, in the form of enucleation of the lesion combined with root canal treatment and apicoectomy, was performed. This case underscores the rare occurrence of plasmacytoma in the jaw region and highlights the importance of surgical management in cases where structural damage or functional impairment is present. Further research on novel treatment approaches is also mentioned, including targeted therapies, immunomodulatory agents, and monoclonal antibodies. The patient is currently under the care of a hematologist for further investigation and the choice of the most appropriate therapy.
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Open AccessCase Report
Cutaneous T-Cell Lymphoma (CTCL) Arising Post Kidney Transplant: A Review of Clinical Variants in the Literature
Hematol. Rep. 2024, 16(1), 11-21; https://doi.org/10.3390/hematolrep16010002 - 28 Dec 2023
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Post-transplant lymphoproliferative disease is a rare disorder with an annual incidence of 0.5% to 3.7%. Development of this disorder carries with it a poor prognosis. In this report, we describe a rare case of post-transplant primary cutaneous T-cell lymphoma (PT-CTCL) mycosis fungoides stage
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Post-transplant lymphoproliferative disease is a rare disorder with an annual incidence of 0.5% to 3.7%. Development of this disorder carries with it a poor prognosis. In this report, we describe a rare case of post-transplant primary cutaneous T-cell lymphoma (PT-CTCL) mycosis fungoides stage IIB in a patient following kidney transplantation, as well as a review of PT-CTCL reported in the literature. The treatment following diagnosis included bexarotene, cyclosporine, and prednisone. Currently, the patient is free from disease. This information aims to add to the knowledge of the prevalence and management of PT-CTCL.
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Open AccessArticle
Feasibility of Quality of Life Assessment in Patients with Lymphoma Aged ≥80 Years Receiving Reduced-Intensity Chemotherapy: A Single-Institute Study
Hematol. Rep. 2024, 16(1), 1-10; https://doi.org/10.3390/hematolrep16010001 - 22 Dec 2023
Abstract
Quality of life (QOL) must be carefully monitored in older patients with lymphoma who are suitable for chemotherapy, but few reports have assessed QOL in older patients who received reduced-intensity chemotherapy. This study investigated QOL in patients with lymphoma aged ≥80 years to
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Quality of life (QOL) must be carefully monitored in older patients with lymphoma who are suitable for chemotherapy, but few reports have assessed QOL in older patients who received reduced-intensity chemotherapy. This study investigated QOL in patients with lymphoma aged ≥80 years to clarify the feasibility of such assessments following reduced-intensity chemotherapy. QOL was prospectively analyzed (using the QOL Questionnaire for Cancer Patients Treated with Anticancer Drugs (QOL-ACD)] and the SF-36®, a comprehensive survey of patient health) among 13 patients (seven women) aged ≥80 years with lymphoma who received reduced-intensity chemotherapy at 4-week intervals at Kyushu University Beppu Hospital between June 2022 and August 2023. Patients were assessed at baseline, in the middle of the protocol, at the end of the protocol, and 6 months after the end of the protocol. The overall response rate was 69%. Almost all severe adverse events (10 patients) occurred during early cycles (cycles 1–2). Common adverse events included hematological toxicities such as neutropenia (10 patients). The daily activity (p = 0.048) and social attitude (p = 0.027) scores of the QOL-ACD and the general health perception (p = 0.044) and social functioning (p = 0.030) scores of the SF-36® were significantly improved during and after chemotherapy. Reduced-dose chemotherapy, if implemented before treatment selection, might permit evaluations of QOL in older patients aged ≥80 years; further investigation is warranted.
Full article
(This article belongs to the Special Issue Personalized Therapies and Clinical Outcomes for Older Patients with Lymphoma)
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Open AccessArticle
Humoral and Cell-Mediated Responses to SARS-CoV-2 Vaccination in a Cohort of Immunodeficient Patients
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Hematol. Rep. 2023, 15(4), 707-716; https://doi.org/10.3390/hematolrep15040071 - 08 Dec 2023
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This study delves into the intricate landscape of SARS-CoV-2 vaccine response in immunodeficient patients, focusing on the dynamics of both humoral and cell-mediated immunity. The cohort includes patients with common variable immunodeficiency (CVI), agammaglobulinemia (XLA), and combined immunodeficiency (CI). The findings reveal varying
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This study delves into the intricate landscape of SARS-CoV-2 vaccine response in immunodeficient patients, focusing on the dynamics of both humoral and cell-mediated immunity. The cohort includes patients with common variable immunodeficiency (CVI), agammaglobulinemia (XLA), and combined immunodeficiency (CI). The findings reveal varying degrees of antibody production, with XLA patients exhibiting no measurable response but displaying a robust T-cell-mediated response. The study emphasizes the importance of considering both arms of the immune system in assessing vaccine immunogenicity, particularly in the context of immunodeficiency. The results challenge conventional measures of vaccine efficacy only based on antibody titers, highlighting the need for a more comprehensive understanding of the immune response in this vulnerable population. This research contributes valuable insights to guide clinical decisions regarding vaccination strategies, booster doses, and overall protection in immunodeficient individuals.
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Open AccessReview
Blastic Plasmocytoid Dendritic Cell Neoplasm (BPDCN): Clinical Features and Histopathology with a Therapeutic Overview
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Hematol. Rep. 2023, 15(4), 696-706; https://doi.org/10.3390/hematolrep15040070 - 08 Dec 2023
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Blastic Plasmacytoid Dendritic Cell Neoplasms (BPDCNs) are a rare, highly aggressive hematological malignant neoplasm that primarily involve the skin, bone marrow, lymph nodes and even extra-nodal sites. The rarity and relative poor description of cases in the literature make it necessary to review
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Blastic Plasmacytoid Dendritic Cell Neoplasms (BPDCNs) are a rare, highly aggressive hematological malignant neoplasm that primarily involve the skin, bone marrow, lymph nodes and even extra-nodal sites. The rarity and relative poor description of cases in the literature make it necessary to review and further studies that deeply investigate this entity not only in a histopathological but also molecular field. In August–September 2023, we searched MEDLINE, PubMed and Scopus for randomized controlled trials (RCTs), narrative and systematic reviews, meta-analyses, observational studies (either longitudinal or retrospective), and case series published in English in the last 25 years using the keywords BPDCN, PDCs, Blastic NK-cell lymphoma, agranular CD4+ NK leukemia/lymphoma, agranular CD4+ CD56+ hematodermic neoplasm/tumor. Despite the progress made in recent years in the diagnosis and biological understanding of the disease, until 2018 there was no clear consensus regarding its treatment and the main therapeutic schemes used were based on chemotherapy regimens already used in the treatment of lymphomas, acute lymphoblastic leukemia (ALL) and/or acute myeloid leukemia (AML). In this narrative review, we address the definition and epidemiological features of BPDCN, provide the different theories on the etiopathogenesis with particular attention to the presumed cell of origin, discuss the main clinical manifestations that provide a sign of its presence, summarize the main histopathological and immunophenotypic characteristics with special attention to the most important markers, and finally, we provide some of the most effective information on the therapeutic treatment modalities of BPDCN.
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Open AccessArticle
Platelet Microvesicles, Inflammation, and Coagulation Markers: A Pilot Study
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Hematol. Rep. 2023, 15(4), 684-695; https://doi.org/10.3390/hematolrep15040069 - 04 Dec 2023
Abstract
Background: Platelet “Microvesicles” (MVs) are studied for their role in blood coagulation and inflammation. The study aimed to establish if MVs are related to age, plasma levels of inflammation, coagulation, and fibrinolysis markers in healthy individuals. Methods: We prospectively enrolled volunteers aged over
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Background: Platelet “Microvesicles” (MVs) are studied for their role in blood coagulation and inflammation. The study aimed to establish if MVs are related to age, plasma levels of inflammation, coagulation, and fibrinolysis markers in healthy individuals. Methods: We prospectively enrolled volunteers aged over 18 years. MVs, plasma levels of C-reactive protein (CRP), Interleukin 6 (IL-6), Interleukin 10 (IL-10), Interleukin 17 (IL-17), and transforming growth factor β (TGF-β), fibrinogen, plasminogen activator inhibitor-1 (PAI-1), von Willebrand factor (VWF), homocysteine, factor VII (FVII), thrombin activatable fibrinolysis inhibitor (TAFI), and Protein S were tested. Results: A total of 246 individuals (median age 65 years (“IQR”54–72)) were evaluated. Both univariate analysis and logistic regression models showed that MVs positively correlate with age, CRP, IL-6, IL-10, IL-17, TGF-β, fibrinogen, PAI-1, VWF, FVII, and homocysteine, while inversely correlating with TAFI and Protein S. The ROC curve analysis performed to identify a cut off for MV values (700 kMP) showed a good accuracy with over-range cytokines fibrinolysis factor and coagulation markers. Conclusions: To the best of our knowledge, this study is the first to correlate MVs with an entire panel of cardiovascular risk factors in healthy individuals. A future possible role of MVs in screening exams is suggested.
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(This article belongs to the Special Issue Personalized Therapy and Clinical Outcomes for Congenital and Acquired Haemorrhagic Disorders, Thromboembolic Disease and Platelet Disorders)
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Open AccessReview
Low-Intensity and Chemo-Free Treatments in Ph+ ALL: Progression-Free Survival Based on Indirect Comparisons
Hematol. Rep. 2023, 15(4), 670-683; https://doi.org/10.3390/hematolrep15040068 - 26 Nov 2023
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In Philadelphia chromosome-positive B-cell (Ph+) acute lymphoblastic leukemia (LLA), growing evidence has accumulated regarding the efficacy of low-intensity and chemo-free regimens. Our objective was to analyze all recent trials evaluating these treatments and to compare them in terms of efficacy. We applied the
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In Philadelphia chromosome-positive B-cell (Ph+) acute lymphoblastic leukemia (LLA), growing evidence has accumulated regarding the efficacy of low-intensity and chemo-free regimens. Our objective was to analyze all recent trials evaluating these treatments and to compare them in terms of efficacy. We applied the Shiny method, an artificial intelligence technique, to analyze Kaplan–Meier curves and reconstruct patient-level data. Reconstructed patient data were then evaluated through standard survival statistics and subjected to indirect head-to-head treatment comparisons. The endpoint was progression-free survival (PFS). Based on 432 reconstructed patients, eight trials were analyzed. The survival data from these trials were pooled into three types of treatments: (i) treatments based on tyrosine kinase inhibitors (TKIs) combined with reduced-intensity chemotherapy (denoted as TKICHE); (ii) TKIs associated with steroids with no chemotherapy (TKISTE); (iii) chemotherapy-free combinations of blinatumomab plus TKIs (TKIBLI). According to the Shiny method, the three PFS curves were reported in a single Kaplan–Meier graph and subjected to survival statistics. In terms of PFS, TKIBLI ranked first, TKICHE second, and TKISTE third; the differences between these three regimens were statistically significant. This multi-treatment Kaplan–Meier graph, generated through the Shiny method, summarized the current evidence on these treatments in both qualitative and quantitative terms.
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Open AccessCase Report
Angioimmunoblastic T-Cell Lymphoma after Treatment of Classic Hodgkin Lymphoma: A Case Report
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Hematol. Rep. 2023, 15(4), 662-669; https://doi.org/10.3390/hematolrep15040067 - 25 Nov 2023
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We report a case of a 24-year-old man who developed angioimmunoblastic T-cell lymphoma (AITL) after treatment for refractory lymphocyte-rich classic Hodgkin lymphoma (LR-CHL). This patient was treated with the BV+AVD (brentuximab vedotin, doxorubicin, vinblastine, and dacarbazine) protocol for LR-CHL but progressed before completing
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We report a case of a 24-year-old man who developed angioimmunoblastic T-cell lymphoma (AITL) after treatment for refractory lymphocyte-rich classic Hodgkin lymphoma (LR-CHL). This patient was treated with the BV+AVD (brentuximab vedotin, doxorubicin, vinblastine, and dacarbazine) protocol for LR-CHL but progressed before completing chemotherapy. The pathological imaging showed the typical findings of LR-CHL at the first onset and first progression. Rescue chemotherapy and high-dose chemotherapy combined with autologous hematopoietic stem cell transplantation (AHSCT) were performed for refractory LR-CHL, and complete remission was achieved. However, the recurrence was suspected 6 months after AHSCT. The pathological findings of the lymph node biopsy at this time were different from those of the previous two lymph node biopsies, demonstrating findings of AITL. The finding of the immunohistochemical staining and polymerase chain reaction results supported the diagnosis. Although it has been reported that the risk for the development of non-Hodgkin lymphoma after treatment for Hodgkin lymphoma is increased, most are B-cell lymphomas, and few cases of AITL have been reported. AITL is a type of peripheral T-cell lymphoma that generally occurs in middle-aged and elderly people and that rarely occurs in young people. Here, we were able to make an accurate diagnosis by performing re-examination even when recurrence of LR-CHL was suspected. As there are no detailed case reports of AITL developing into secondary non-Hodgkin lymphoma, here we report on an identified case.
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Open AccessArticle
Red Cell Distribution Width and Prediabetes in Adults in Northern Sudan: A Case–Control Study
Hematol. Rep. 2023, 15(4), 651-661; https://doi.org/10.3390/hematolrep15040066 - 20 Nov 2023
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Diabetes mellitus (DM) is a major public health issue worldwide. Red cell distribution width (RDW) has been reported to have predictive value in several diseases, including DM. Few data exist on the association between RDW and the prediabetic stage. Thus, the present study
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Diabetes mellitus (DM) is a major public health issue worldwide. Red cell distribution width (RDW) has been reported to have predictive value in several diseases, including DM. Few data exist on the association between RDW and the prediabetic stage. Thus, the present study aimed to investigate the association between RDW and prediabetes in adults in Sudan. This case–control study was conducted in Northern Sudan in 2022. The cases (n = 107) were prediabetic patients categorized according to the level of glycated hemoglobin (HbA1c), which ranged from 5.7% to 6.4%, while the controls (n = 107) were healthy participants. A questionnaire was used to collect the data. Standard methods were used to measure the HbAIc level and RDW. Logistic regression analysis was performed. The median (interquartile range (IQR)) of the RDW was significantly higher in prediabetic patients than in the controls (14.5% [13.8–15.3%] vs. 14.1% [13.6–14.7%], p = 0.003). Sex, educational level, occupational status, marital status, cigarette smoking, alcohol consumption, family history of DM, and body mass index were not associated with prediabetes. In the multivariate-adjusted model, higher age and higher RDW were associated with prediabetes. A positive correlation was found between RDW and HbA1c levels (r = 0.19, p = 0.006). In conclusion, this study supports the use of RDW as a predictor of DM.
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Open AccessReview
Association of microRNA Polymorphisms with Toxicities Induced by Methotrexate in Children with Acute Lymphoblastic Leukemia
Hematol. Rep. 2023, 15(4), 634-650; https://doi.org/10.3390/hematolrep15040065 - 20 Nov 2023
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Methotrexate (MTX), a structurally related substance to folic acid, is an important chemotherapeutic agent used for decades in the treatment of pediatric acute lymphoblastic leukemia (ALL) and other types of cancer as non-Hodgkin lymphomas and osteosarcomas. Despite the successful outcomes observed, the primary
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Methotrexate (MTX), a structurally related substance to folic acid, is an important chemotherapeutic agent used for decades in the treatment of pediatric acute lymphoblastic leukemia (ALL) and other types of cancer as non-Hodgkin lymphomas and osteosarcomas. Despite the successful outcomes observed, the primary drawback is the variability in the pharmacokinetics and pharmacodynamics between patients. The main adverse events related to its use are nephrotoxicity, mucositis, and myelosuppression, especially when used in high doses. The potential adverse reactions and toxicities associated with MTX are a cause for concern and may lead to dose reduction or treatment interruption. Genetic variants in MTX transport genes have been linked to toxicity. Pharmacogenetic studies conducted in the past focused on single nucleotide polymorphisms (SNPs) in the coding and 5′-regulatory regions of genes. Recent studies have demonstrated a significant role of microRNAs (miRNAs) in the transport and metabolism of drugs and in the regulation of target genes. In the last few years, the number of annotated miRNAs has continually risen, in addition to the studies of miRNA polymorphisms and MTX toxicity. Therefore, the objective of the present study is to investigate the role of miRNA variants related to MTX adverse effects.
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Open AccessCase Report
Composite Angioimmunoblastic T-Cell Lymphoma and Diffuse Large B-Cell Lymphoma Presenting with Distributive Shock
Hematol. Rep. 2023, 15(4), 627-633; https://doi.org/10.3390/hematolrep15040064 - 16 Nov 2023
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Diffuse large B-cell lymphoma (DLBCL) and angioimmunoblastic T-cell lymphoma (AITL) are two subtypes of non-Hodgkin lymphoma (NHL). The simultaneous occurrence of DLBCL and AITL in a composite lymphoma is very rare, and there are no established treatment regimens. We present the case of
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Diffuse large B-cell lymphoma (DLBCL) and angioimmunoblastic T-cell lymphoma (AITL) are two subtypes of non-Hodgkin lymphoma (NHL). The simultaneous occurrence of DLBCL and AITL in a composite lymphoma is very rare, and there are no established treatment regimens. We present the case of an 85-year-old male admitted to the intensive care unit with distributive shock, lymphocytosis, and lymphadenopathy, who was subsequently diagnosed with composite AITL and DLBCL, and treated with brentuximab vedotin (BV) and rituximab. To our knowledge, this is the first case of composite lymphoma presenting with distributive shock and treated with BV and rituximab, with successful resolution of shock.
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Open AccessReview
Chimeric Antigen Receptor T Cell Therapy in Acute Myeloid Leukemia: Trials and Tribulations
Hematol. Rep. 2023, 15(4), 608-626; https://doi.org/10.3390/hematolrep15040063 - 12 Nov 2023
Abstract
Acute myeloid leukemia (AML) is a heterogeneous hematological malignancy that is often associated with relapse and drug resistance after standard chemotherapy or targeted therapy, particularly in older patients. Hematopoietic stem cell transplants are looked upon as the ultimate salvage option with curative intent.
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Acute myeloid leukemia (AML) is a heterogeneous hematological malignancy that is often associated with relapse and drug resistance after standard chemotherapy or targeted therapy, particularly in older patients. Hematopoietic stem cell transplants are looked upon as the ultimate salvage option with curative intent. Adoptive cell therapy using chimeric antigen receptors (CAR) has shown promise in B cell malignancies and is now being investigated in AML. Initial clinical trials have been disappointing in AML, and we review current strategies to improve efficacy for CAR approaches. The extensive number of clinical trials targeting different antigens likely reflects the genetic heterogeneity of AML. The limited number of patients reported in multiple early clinical studies makes it difficult to draw conclusions about CAR safety, but it does suggest that the efficacy of this approach in AML lags behind the success observed in B cell malignancies. There is a clear need not only to improve CAR design but also to identify targets in AML that show limited expression in normal myeloid lineage cells.
Full article
(This article belongs to the Special Issue Cell Therapies in Hematology: Current Updates and Perspectives)
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Open AccessArticle
Management and Outcomes of Invasive Procedures in Individuals with Hemophilia A on Emicizumab Prophylaxis: A Single Center Experience
Hematol. Rep. 2023, 15(4), 597-607; https://doi.org/10.3390/hematolrep15040062 - 01 Nov 2023
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Prophylactic treatment with emicizumab has become an important and effective bleeding prevention for people with hemophilia A (PwHA). Perioperative management of PwHA using emicizumab prophylaxis is still challenging due to a lack of experience. Medical records of perioperative management and outcomes were reviewed,
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Prophylactic treatment with emicizumab has become an important and effective bleeding prevention for people with hemophilia A (PwHA). Perioperative management of PwHA using emicizumab prophylaxis is still challenging due to a lack of experience. Medical records of perioperative management and outcomes were reviewed, and data were collected for adult PwHA receiving emicizumab and undergoing surgical procedures between August 2019 and July 2022 at the University Medical Center Ljubljana. Twelve surgical procedures were performed in eight PwHA (one with FVIII inhibitors) while on emicizumab prophylaxis. Three minor procedures included cataract surgery, cystoscopic lithotripsy, and percutaneous coronary intervention. Nine major surgeries included four osteosyntheses, necrectomy of chronic osteomyelitis with new ankle arthrodesis, two below-knee amputations, total knee replacement, and placement of ventriculostomy after a spontaneous intraventricular hemorrhage. No major bleeds, thrombotic events or deaths, or new inhibitors appeared. Our real-world experience demonstrates that minor and major surgeries can be performed safely in PwHA on emicizumab prophylaxis. Additional data are needed to optimize dosing/duration of additional hemostatic agents in diverse invasive procedures and complex clinical situations.
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Open AccessCase Report
A Push to Consider Mantle Cell Lymphoma in Adults with Leukemia/Lymphoma with Blastoid Morphology
Hematol. Rep. 2023, 15(4), 592-596; https://doi.org/10.3390/hematolrep15040061 - 13 Oct 2023
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Mantle cell lymphoma (MCL) is an intermediate-grade B-cell lymphoma, representing 2.8% of all non-Hodgkin lymphomas in the US. It is associated with t(11;14)(q13; q23), which leads to the overexpression of cyclin D1, consequently promoting cell proliferation. MCL usually expresses CD19, CD20, CD43, surface
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Mantle cell lymphoma (MCL) is an intermediate-grade B-cell lymphoma, representing 2.8% of all non-Hodgkin lymphomas in the US. It is associated with t(11;14)(q13; q23), which leads to the overexpression of cyclin D1, consequently promoting cell proliferation. MCL usually expresses CD19, CD20, CD43, surface immunoglobulins, FMC7, BCL2, cyclin D1, CD5, and SOX11. Herein is a case of a 67-year-old male, referred to our facility with shortness of breath, anemia (hemoglobin of 5.3 g/dL), thrombocytopenia (12 × 109/L), and leukocytosis (283 × 109/L). A peripheral blood smear showed marked lymphocytosis with blastoid morphology. Morphologic examination of the bone marrow biopsy revealed a diffuse sheet of blastoid cells expressing CD20 and CD10, but without CD5 or cyclin D1. Given these features, a differential diagnosis of diffuse large B-cell lymphoma (DLBCL) with germinal center derivation, high-grade follicular lymphoma, and Burkitt lymphoma was considered, with the latter not favored due to morphology. Additional studies revealed positive SOX11, and fluorescence in situ hybridization (FISH) studies detected t(11;14). These additional studies supported diagnosis of the blastoid variant of MCL. In conclusion, we present a unique and challenging case of MCL without cyclin D1 or CD5, but with an expression of CD10 and SOX11, along with t(11;14). Pathologists should explicitly consider the blastoid variant of MCL when dealing with mature B-cell neoplasms with blastoid morphology in adults, and utilize a broad panel of ancillary studies, including FISH and SOX11.
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Open AccessArticle
Analysis of Costs per Responder in US Adults with Paroxysmal Nocturnal Hemoglobinuria with a Suboptimal Response to Prior Eculizumab Treatment
Hematol. Rep. 2023, 15(4), 578-591; https://doi.org/10.3390/hematolrep15040060 - 13 Oct 2023
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European Society for Blood and Marrow Transplantation (EBMT) hematologic response categories comprehensively assess complement inhibitor responses in patients with paroxysmal nocturnal hemoglobinuria (PNH). Using data from the 16-week randomized controlled period of the phase 3 PEGASUS trial (N = 80), we estimated the
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European Society for Blood and Marrow Transplantation (EBMT) hematologic response categories comprehensively assess complement inhibitor responses in patients with paroxysmal nocturnal hemoglobinuria (PNH). Using data from the 16-week randomized controlled period of the phase 3 PEGASUS trial (N = 80), we estimated the treatment cost per responder by the EBMT response category for pegcetacoplan and eculizumab in adults with PNH and a suboptimal response to eculizumab. Average drug costs per responder, number needed to treat, and incremental drug costs per responder were estimated using dosages administered during the trial (base case). A US payer perspective (2020 US dollars) was used. Scenario analyses were conducted for various costs, dosages, treatment durations, patient populations, and settings. In total, 30 of 41 (73%) who switched to pegcetacoplan and 2 of 39 (5%) patients who continued eculizumab had a good, major, or complete response (good-to-complete responders) at Week 16. Average weekly drug costs per good-to-complete responder were USD 15,923 with pegcetacoplan and USD 216,100 with eculizumab; average weekly drug costs per patient were USD 11,651 and USD 11,082, respectively. Average drug costs per good-to-complete responder with pegcetacoplan were similar across complement inhibitor-naïve populations and were consistently lower than with eculizumab. Switching from eculizumab to pegcetacoplan allowed more patients with a suboptimal response to attain a good-to-complete response at lower costs. These results apply to patients with a suboptimal response to prior eculizumab treatment only.
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Open AccessReview
A Review of Hematological Complications and Treatment in COVID-19
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Hematol. Rep. 2023, 15(4), 562-577; https://doi.org/10.3390/hematolrep15040059 - 13 Oct 2023
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COVID-19, caused by SARS-CoV-2, and its variants have spread rapidly across the globe in the past few years, resulting in millions of deaths worldwide. Hematological diseases and complications associated with COVID-19 severely impact the mortality and morbidity rates of patients; therefore, there is
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COVID-19, caused by SARS-CoV-2, and its variants have spread rapidly across the globe in the past few years, resulting in millions of deaths worldwide. Hematological diseases and complications associated with COVID-19 severely impact the mortality and morbidity rates of patients; therefore, there is a need for oversight on what pharmaceutical therapies are prescribed to hematologically at-risk patients. Thrombocytopenia, hemoglobinemia, leukopenia, and leukocytosis are all seen at increased rates in patients infected with COVID-19 and become more prominent in patients with severe COVID-19. Further, COVID-19 therapeutics may be associated with hematological complications, and this became more important in immunocompromised patients with hematological conditions as they are at higher risk of hematological complications after treatment. Thus, it is important to understand and treat COVID-19 patients with underlying hematological conditions with caution. Hematological changes during COVID-19 infection and treatment are important because they may serve as biomarkers as well as to evaluate the treatment response, which will help in changing treatment strategies. In this literature review, we discuss the hematological complications associated with COVID-19, the mechanisms, treatment groups, and adverse effects of commonly used COVID-19 therapies, followed by the hematological adverse events that could arise due to therapeutic agents used in COVID-19.
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Open AccessCase Report
Transient Leukemoid Reaction from T-Cell Large Granular Lymphocytes Post Autologous Stem Cell Transplant in a Patient Affected by Hodgkin Lymphoma
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Hematol. Rep. 2023, 15(4), 555-561; https://doi.org/10.3390/hematolrep15040058 - 11 Oct 2023
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Monoclonal T-cell lymphocytosis has been reported in patients with concomitant autoimmune diseases, viral infections, or immunodeficiencies. Referred to as T-cell large granular lymphocytic leukemia (T-LGLL), most cases cannot identify the triggering cause. Only small case series have been reported in the literature, and
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Monoclonal T-cell lymphocytosis has been reported in patients with concomitant autoimmune diseases, viral infections, or immunodeficiencies. Referred to as T-cell large granular lymphocytic leukemia (T-LGLL), most cases cannot identify the triggering cause. Only small case series have been reported in the literature, and no treatment consensus exists. T-cell lymphocytosis may also appear after the transplant of hematopoietic stem cells or solid organs. Rare cases have been reported in patients undergoing autologous stem cell transplant (ASCT) for hematological diseases (including multiple myeloma or non-Hodgkin’s lymphoma). Here, we describe the singular case of a patient who underwent ASCT for Hodgkin’s lymphoma and displayed the onset of T-LGLL with an uncommonly high number of lymphocytes in peripheral blood and their subsequent spontaneous remission.
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Changes in Hematological and Hemorheological Parameters Following Mild COVID-19: A 4-Month Follow-Up Study
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, , , , , , and
Hematol. Rep. 2023, 15(4), 543-554; https://doi.org/10.3390/hematolrep15040057 - 10 Oct 2023
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Background: Coronavirus Disease 2019 (COVID-19) was described to affect red blood cells (RBC) in both severe and mild disease courses. The aim of this study was to investigate whether hematological and hemorheological changes that were previously described for COVID-19 patients after the acute
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Background: Coronavirus Disease 2019 (COVID-19) was described to affect red blood cells (RBC) in both severe and mild disease courses. The aim of this study was to investigate whether hematological and hemorheological changes that were previously described for COVID-19 patients after the acute infection state are still prominent after another 4 months to assess potential long-term effects. Methods: Hematological and RBC rheological parameters, including deformability and aggregation, were measured 41 days after infection in COVID-19 patients and non-COVID control (T0) and 4 months later in COVID-19 patients (T1). Results: The data confirm alterations in hematological parameters, mainly related to cell volume and hemoglobin concentration, but also reduced deformability and increased aggregation at T0 compared to control. While RBC deformability seems to have recovered, hemoglobin-related parameters and RBC aggregation were still impaired at T1. The changes were thus more pronounced in male COVID-19 patients. Conclusion: COVID-19-related changes of the RBC partly consist of several months and might be related to persistent symptoms reported by many COVID-19 patients.
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Real-World Clinical Characterisation of Polycythaemia Vera Patients from a Prospective Registry in Portugal: Is Resistance to Hydroxyurea a Reality?
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Hematol. Rep. 2023, 15(3), 532-542; https://doi.org/10.3390/hematolrep15030056 - 13 Sep 2023
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Patients with polycythaemia vera (PV) are at increased risk of thrombosis and haemorrhages. Although hydroxyurea (HU) has been the frontline therapy for patients at high risk of vascular complications, about 25% of patients develop resistance/intolerance to this therapy. The aim of this non-interventional,
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Patients with polycythaemia vera (PV) are at increased risk of thrombosis and haemorrhages. Although hydroxyurea (HU) has been the frontline therapy for patients at high risk of vascular complications, about 25% of patients develop resistance/intolerance to this therapy. The aim of this non-interventional, multicentre cohort study was to understand the clinical characteristics and HU treatment response of Portuguese PV patients. HU resistance/intolerance was defined according to adjusted European LeukemiaNet (ELN) criteria. In total, 134 PV patients with a mean (SD) disease duration of 4.8 (5.0) years were included and followed up for 2 years. At baseline, most patients were ≥60 years old (83.2%), at high risk for thrombotic events (87.2%), and receiving HU therapy (79.1%). A total of 10 thrombotic events and 8 haemorrhagic events were reported, resulting in a 5-year probability of thrombo-haemorrhagic events of 17.2%. Haematocrit (p = 0.007), haemoglobin (p = 0.012) and MPN10 symptom score (12.0 (11.6) vs. 10.3 (9.1); p = 0.041) decreased significantly at the 24-month visit compared to baseline. Overall, 75.9% of patients met at least one of the adjusted ELN criteria for HU resistance, and 14.4% of patients remained on HU throughout the study. The results from this real-world study may help identify the subset of patients at higher risk for disease sequelae who may benefit from earlier second-line treatment.
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