Hematology Reports

12 pages, 1228 KiB

Open AccessArticle

Clinical Manifestations, Prognostic Factors, and Outcomes of Extranodal Natural Killer T-Cell Lymphoma: A Single-Center Experience in Thailand

by Wasinee Kaewboot, Lalita Norasetthada, Adisak Tantiworawit, Chatree Chai-Adisaksopha, Sasinee Hantrakool, Thanawat Rattanathammethee, Pokpong Piriyakhuntorn, Nonthakorn Hantrakun, Teerachat Punnachet and Ekarat Rattarittamrong

Hematol. Rep. 2024, 16(4), 769-780; https://doi.org/10.3390/hematolrep16040073 - 29 Nov 2024

Abstract

Background/Objectives: The primary objective of this study was to investigate clinical manifestations, time to diagnosis, and number of biopsies in patients with extranodal natural killer T-cell lymphoma (ENKTL). The secondary objectives were to determine response rates, survival outcomes, prognostic factor for overall [...] Read more.

Background/Objectives: The primary objective of this study was to investigate clinical manifestations, time to diagnosis, and number of biopsies in patients with extranodal natural killer T-cell lymphoma (ENKTL). The secondary objectives were to determine response rates, survival outcomes, prognostic factor for overall survival (OS), and validation of the Prognostic Index of Natural Killer Lymphoma (PINK), Ann Arbor staging system (AASS), and the CA system. Methods: This retrospective study included data pertaining to patients with newly diagnosed ENKTL in Chiang-Mai University Hospital from 2004 to 2020. Comparisons between the areas under the receiver operating characteristic curve (AUC) of prognostic models (PINK, AASS, and CA system) were made. Results: Sixty patients were enrolled (n = 60) with a mean age of 49.1 ± 13.4 years. The most frequent symptom of ENKTL was nasal obstruction (66%). The median time to diagnosis was 22 days (ranging from 3 to 84 days), with 36.7% requiring more than one biopsy for diagnosis. Most patients presented with limited stage disease (75%). The median OS was 49 months. Factors associated with increased mortality were advanced stage, bone marrow involvement, gastrointestinal tract involvement, and receiving chemotherapy. Following prognostic model validation, the CA system model scored the highest level of accuracy (AUC 0.61), followed by AASS (AUC 0.58) and PINK (AUC 0.54). Conclusions: Patients with ENKTL commonly presented with nasal obstruction, with 36.7% requiring more than one biopsy for diagnosis. An advanced stage, bone marrow involvement, or gastrointestinal tract involvement were associated with poor OS. The CA system model has the highest level of accuracy for prognostic determination. Full article

► Show Figures

Figure 1

Figure 1
Kaplan–Meier curves showing overall survival according to the stage of disease. Full article ">Figure 2
Kaplan–Meier curves showing overall survival according to modified AASS. Full article ">Figure 3
Kaplan–Meier curves showing overall survival according to PINK. Full article ">Figure 4
Kaplan–Meier curves showing overall survival according to CA staging system. Full article ">Figure 5
ROC analysis of modified AASS ((A): Blue line), PINK ((B): Blue line), CA system ((C): Blue line), and all models (D). The green lines in subfigure (A–C) represent reference. Full article ">Figure 6
Overall survival of patients with limited stage disease treated with combined chemoradiotherapy (red line) and chemotherapy or radiotherapy alone (blue line). Full article ">Figure 7
Overall survival of patients with limited stage disease according to L-asparaginase-based regimen. Full article ">Figure 8
Overall survival of patients with advanced stage disease according to L-asparaginase-based regimen. Full article ">

17 pages, 753 KiB

Open AccessSystematic Review

The Role of 11C-Methionine PET Imaging for the Evaluation of Lymphomas: A Systematic Review

by Francesco Dondi, Maria Gazzilli, Gian Luca Viganò, Antonio Rosario Pisani, Cristina Ferrari, Giuseppe Rubini and Francesco Bertagna

Hematol. Rep. 2024, 16(4), 752-768; https://doi.org/10.3390/hematolrep16040072 - 27 Nov 2024

Abstract

Background: In the last years, different evidence has underlined a possible role for [11C]-methionine ([11C]MET) positron emission tomography (PET) imaging for the evaluation of lymphomas. The aim of this paper was, therefore, to review the available scientific literature focusing on this topic. [...] Read more.

Background: In the last years, different evidence has underlined a possible role for [11C]-methionine ([11C]MET) positron emission tomography (PET) imaging for the evaluation of lymphomas. The aim of this paper was, therefore, to review the available scientific literature focusing on this topic. Methods: A wide literature search of the PubMed/MEDLINE, Scopus and Cochrane Library databases was conducted in order to find relevant published articles investigating the role of [11C]MET in the assessment of lymphomas. Results: Eighteen studies were included in the systematic review and the main fields of application of this imaging modality were the evaluation of disease, therapy response assessment, prognostic evaluation and differential diagnosis with other pathological conditions. Conclusion: Even with heterogeneous evidence, a possible role for [11C]MET PET imaging in the assessment of lymphomas affecting both the whole body and the central nervous system was underlined. When compared to [18F]fluorodesoxyglucose ([18F]FDG) imaging, in general, similar results have been reported between the two modalities in these settings. Full article

► Show Figures

Figure 1

10 pages, 435 KiB

Open AccessArticle

Serum Vitamin D in Children with Hemophilia A and Its Association with Joint Health and Quality of Life

by Aida M. S. Salem, Takwa Mohamed AbdEltwwab, Hanan Hosni Moawad, Marwa O. Elgendy, Reham S. Al-Fakharany, Ahmed Khames and Mohamed Hussein Meabed

Hematol. Rep. 2024, 16(4), 742-751; https://doi.org/10.3390/hematolrep16040071 - 26 Nov 2024

Abstract

Background/Objectives: Hemophilia A is an X-linked recessive illness produced by a deficiency of coagulation factor VIII. This study aimed to evaluate serum vitamin D in hemophilic pediatric patients and its correlation with joint health and quality of life. Methods: This case-control [...] Read more.

Background/Objectives: Hemophilia A is an X-linked recessive illness produced by a deficiency of coagulation factor VIII. This study aimed to evaluate serum vitamin D in hemophilic pediatric patients and its correlation with joint health and quality of life. Methods: This case-control study was performed on ninety children under the age of 18 years old and separated into two groups: study group of 45 children with hemophilia A and control group of 45 healthy children at an outpatient pediatric hematology clinic at the Beni-Suef University hospitals. Results: Serum vitamin D levels were significantly lower in hemophilia A patients than in controls (p < 0.001). The level of serum vitamin D was deficient in 38 (84.4%), insufficient in 4 (8.8%) and sufficient in 3 (6.6%) in the study group while deficient in 8 (17.7%), insufficient in 16 (35.5%) and sufficient in 21 (46.6%) in the control group. Total hemophilia joint health score (HJHS) had a significant negative correlation with serum total calcium (R = −0.31, p = 0.038) and serum vitamin D level (R = −0.974, p < 0.001) while also positively correlated with alkaline phosphatase (R = 0.834, p < 0.001). A quality-of-life index that is specific to total hemophilia (Haemo-Qol/Haem-A-QoL) had a significant positive correlation with total hemophilia joint health score (HJHS) (R = 0.934, p < 0.001) and negatively correlated with serum vitamin D level (R = −0.924, p-value lower than 0.001), alkaline phosphatase (R = 0.842, p < 0.001), and severity of hemophilia (R = 0.67, p < 0.001). Conclusions: patients with hemophilia A had lower vitamin D levels than healthy controls. The severity of vitamin D deficiency is related positively to (HJHS) hemophilia and quality of life hemophilia cases according to Haemo-QoL. Full article

► Show Figures

Figure 1

10 pages, 3590 KiB

Open AccessReview

Mesenchymal Stem Cells and Reticulated Platelets: New Horizons in Multiple Myeloma

by Cristian Alejandro Mera Azaín, Johan Leandro Vargas Pasquel, Sandra Milena Quijano Gómez and Viviana Marcela Rodríguez-Pardo

Hematol. Rep. 2024, 16(4), 732-741; https://doi.org/10.3390/hematolrep16040070 - 23 Nov 2024

Abstract

Multiple myeloma (MM) is a malignant plasma cell disorder characterized by the accumulation of abnormal plasma cells in the bone marrow. Mesenchymal stem cells (MSCs) and reticulated platelets (RPs) have been implicated in the pathogenesis of MM. This narrative review aims to explore [...] Read more.

Multiple myeloma (MM) is a malignant plasma cell disorder characterized by the accumulation of abnormal plasma cells in the bone marrow. Mesenchymal stem cells (MSCs) and reticulated platelets (RPs) have been implicated in the pathogenesis of MM. This narrative review aims to explore the role of MSCs and RPs in the pathophysiology of MM, particularly their clinical use as possible variables of prognostic value in this hematologic neoplasia. The interaction between MSCs and MM cells within the bone marrow microenvironment supports MM cell survival, proliferation, and drug resistance. MSCs contribute to the development and maintenance of MM through the secretion of various factors, including cytokines, chemokines, and growth factors. Moreover, RPs, young and highly reactive platelets, have been implicated in promoting angiogenesis, tumor growth, and metastasis in MM. Several studies show that cells such as MSCs and platelets participate actively in the biology of the disease. Still, in clinical practice, they are not considered part of evaluating affected patients. In this review, we explore the possibility of including the evaluation of MSCs and PRs in the clinical practice for patients with MM as part of the strategies to improve the outcomes of this disease. Full article

► Show Figures

Figure 1

8 pages, 5591 KiB

Open AccessCase Report

A Case of B-Cell Lymphoblastic Lymphoma Presenting with an Isolated Epidural Mass Treated Successfully with Radiotherapy Followed by United Kingdom Acute Lymphoblastic Leukemia (UKALL) Chemotherapy Protocol

by Musa Fares Alzahrani

Hematol. Rep. 2024, 16(4), 724-731; https://doi.org/10.3390/hematolrep16040069 - 23 Nov 2024

Abstract

Background: B-cell lymphoblastic lymphoma (B-LBL) is an aggressive type of non-Hodgkin lymphoma that usually involves lymph nodes, skin and soft tissue. Bone marrow and peripheral blood are normally spared from involvement in the disease. B-LBL typically forms solid masses that have similar pathologic [...] Read more.

Background: B-cell lymphoblastic lymphoma (B-LBL) is an aggressive type of non-Hodgkin lymphoma that usually involves lymph nodes, skin and soft tissue. Bone marrow and peripheral blood are normally spared from involvement in the disease. B-LBL typically forms solid masses that have similar pathologic and immunophenotypic features to their liquid counterpart, B-cell acute lymphoblastic leukemia (B-ALL). The presentation of B-LBL with a solitary epidural mass at the cervical spine is very rare and the optimal treatment of such cases is unknown. Most of the literature on the management of B-LBL comes from small case series, pediatric patients, or as part of retrospective data that combine B-LBL with B-ALL cases. Case presentation: The case presented herein is a unique presentation that was treated using three modalities, namely surgical resection, radiotherapy and consolidation with systemic chemotherapy, adopted from the United Kingdom acute lymphoblastic leukemia (UKALL14) protocol. Conclusions: The patient attained complete remission following the planned treatment and is still in remission for more than four and half years from the time of his initial diagnosis. Full article

► Show Figures

Figure 1

Figure 1
MRI of the cervical spine showed that there is an intraspinal epidural mass (red arrows) measuring 1.2 × 1.8 × 3.2 mm (AP, TV and CC diameters) in the ventrolateral aspect at the level of C3–C4 extending to the left neural foramen, impinging the adjacent nerve root and compressing the spinal cord with cervical myelopathy. The mass demonstrates avid homogeneous enhancement in postcontrast study and DWI restriction. Abbreviation: Magnetic resonance imaging (MRI), anteroposterior (AP), transverse (TV), and craniocaudal (CC) planes. Full article ">Figure 2
(A) High power microscopic view of lymphoblastic lymphoma. Note the presence of numerous monotonous lymphoid cells showing irregular nuclei, fine nuclear chromatin and conspicuous nucleoli in some cells. [Hematoxylin/Eosin (H/E) stain ×600 magnification (Mag)]. (B) TdT Immunohistochemical stain. Note the strong positive nuclear staining. Mag ×600. (C) Strong positive membranous staining of the lymphoma cells with CD79A confirming the B cell lineage of these cells. Mag ×600. (D) Strong positive cytoplasmatic and membranous staining with CD99 stain. This stain, although not specific, is commonly positive in cases of lymphoblastic lymphoma. Mag ×600. Full article ">Figure 2 Cont.
(A) High power microscopic view of lymphoblastic lymphoma. Note the presence of numerous monotonous lymphoid cells showing irregular nuclei, fine nuclear chromatin and conspicuous nucleoli in some cells. [Hematoxylin/Eosin (H/E) stain ×600 magnification (Mag)]. (B) TdT Immunohistochemical stain. Note the strong positive nuclear staining. Mag ×600. (C) Strong positive membranous staining of the lymphoma cells with CD79A confirming the B cell lineage of these cells. Mag ×600. (D) Strong positive cytoplasmatic and membranous staining with CD99 stain. This stain, although not specific, is commonly positive in cases of lymphoblastic lymphoma. Mag ×600. Full article ">Figure 3
Follow up MRI Showed that there has been interval laminectomy performed at C3–C5 levels, resolution of the large intraspinal anterior epidural mass on the ventrolateral aspect of the canal at the level of C3–C4, improvement of the signal alteration within spinal cord due to cord myelopathy, no significant diffusion restriction, stable faint mild enhancement of C4 vertebra and new alteration on T1 and T2-weighted images at C2 to C6 vertebrae related to radiotherapy. Full article ">

10 pages, 888 KiB

Open AccessArticle

Efficacy of Anti-CD38 Monoclonal Antibodies for Relapsed or Refractory Multiple Myeloma in Stem Cell Transplant-Ineligible Patients Aged over 65 Years: A Propensity Score-Matched Study

by Satoshi Yamasaki, Michitoshi Hashiguchi, Nao Yoshida-Sakai, Hiroto Jojima, Koichi Osaki, Takashi Okamura and Yutaka Imamura

Hematol. Rep. 2024, 16(4), 714-723; https://doi.org/10.3390/hematolrep16040068 - 18 Nov 2024

Abstract

Background: The development of newer agents, including anti-CD38 monoclonal antibodies (mAbs), has significantly improved overall survival (OS) in patients with relapsed or refractory multiple myeloma (RRMM). However, the treatment of older patients with RRMM who are transplant-ineligible remains challenging. Methods: We retrospectively evaluated [...] Read more.

Background: The development of newer agents, including anti-CD38 monoclonal antibodies (mAbs), has significantly improved overall survival (OS) in patients with relapsed or refractory multiple myeloma (RRMM). However, the treatment of older patients with RRMM who are transplant-ineligible remains challenging. Methods: We retrospectively evaluated OS in 78 transplant-ineligible patients with RRMM who were aged ≥ 65 years and treated at our institution between February 2012 and November 2023. Results: Unadjusted OS was significantly longer in the anti-CD38 mAb-exposed group (i.e., those previously treated with daratumumab and receiving isatuximab plus pomalidomide and low-dose dexamethasone because of disease progression during treatment with daratumumab [n = 6], daratumumab plus pomalidomide and low-dose dexamethasone [n = 9], or isatuximab plus pomalidomide and low-dose dexamethasone without daratumumab-exposure [n = 14]) than in the anti-CD38 mAb-naïve group (no exposure to daratumumab or isatuximab [n = 49]) (p < 0.001). To address potential confounder factors associated with use or nonuse of anti-CD38 mAbs, we performed propensity score matching (PSM) using age, sex, performance status, and Geriatric 8 and Instrumental Activities of Daily Living scores. PSM identified 14 subjects from the anti-CD38 mAb-exposed group with baseline characteristics similar to those of 14 subjects from the anti-CD38 mAb-naïve group. After PSM, the adjusted OS was significantly longer in the anti-CD38 mAb-exposed group than in the anti-CD38 mAb-naïve group (p < 0.001). Conclusion: These findings provide insights into the optimal use of anti-CD38 mAbs in patients with RRMM who are transplant-ineligible and aged ≥65 years and on candidates who are appropriate for novel approaches, such as chimeric antigen receptor T-cell or bispecific T-cell engager therapy. Full article

► Show Figures

Figure 1

16 pages, 1143 KiB

Open AccessReview

Treatment Strategies Used in Treating Myelofibrosis: State of the Art

by Massimo Martino, Martina Pitea, Annalisa Sgarlata, Ilaria Maria Delfino, Francesca Cogliandro, Anna Scopelliti, Violetta Marafioti, Simona Polimeni, Gaetana Porto, Giorgia Policastro, Giovanna Utano, Maria Pellicano, Giovanni Leanza and Caterina Alati

Hematol. Rep. 2024, 16(4), 698-713; https://doi.org/10.3390/hematolrep16040067 - 30 Oct 2024

Abstract

Background: Current drug therapy for myelofibrosis does not alter the natural course of the disease or prolong survival, and allogeneic stem cell transplantation is the only curative treatment modality. For over a decade, the Janus kinase (JAK) inhibitor ruxolitinib has been the standard [...] Read more.

Background: Current drug therapy for myelofibrosis does not alter the natural course of the disease or prolong survival, and allogeneic stem cell transplantation is the only curative treatment modality. For over a decade, the Janus kinase (JAK) inhibitor ruxolitinib has been the standard of care. More recently, newer-generation JAK inhibitors have joined the ranks of accepted treatment options. Objectives: The primary goal of treatment is to reduce spleen size and minimize disease-related symptoms. Prognostic scoring systems are used to designate patients as being at lower or higher risk. For transplant-eligible patients, transplant is offered to those with a bridge of a JAK inhibitor; patients who are not eligible for transplant are usually offered long-term therapy with a JAK inhibitor. Limited disease-modifying activity, dose-limiting cytopenias, and other adverse effects have contributed to discontinuation of JAK inhibitor treatment. Conclusions: Novel JAK inhibitors and combination approaches are currently being explored to overcome these shortcomings. Further research will be essential to establish optimal therapeutic approaches in first-line and subsequent treatments. Full article

► Show Figures

Figure 1

16 pages, 1312 KiB

Open AccessReview

Alternative Splicing: A Potential Therapeutic Target in Hematological Malignancies

by Gazmend Temaj, Silvia Chichiarelli, Sarmistha Saha, Pelin Telkoparan-Akillilar, Nexhibe Nuhii, Rifat Hadziselimovic and Luciano Saso

Hematol. Rep. 2024, 16(4), 682-697; https://doi.org/10.3390/hematolrep16040066 - 29 Oct 2024

Abstract

Leukemia represents the most prevalent malignancy in children, constituting 30% of childhood cancer cases, with acute lymphoblastic leukemia (ALL) being particularly heterogeneous. This paper explores the role of alternative splicing in leukemia, highlighting its significance in cancer development and progression. Aberrant splicing is [...] Read more.

Leukemia represents the most prevalent malignancy in children, constituting 30% of childhood cancer cases, with acute lymphoblastic leukemia (ALL) being particularly heterogeneous. This paper explores the role of alternative splicing in leukemia, highlighting its significance in cancer development and progression. Aberrant splicing is often driven by mutations in splicing-factor genes, which can lead to the production of variant proteins that contribute to oncogenesis. The spliceosome, a complex of small nuclear RNAs and proteins, facilitates RNA splicing, a process critical for generating diverse mRNA and protein products from single genes. Mutations in splicing factors, such as U2AF1, SF3B1, SRSF2, ZRSR2, and HNRNPH1, are frequently observed across various hematological malignancies and are associated with poor prognosis and treatment resistance. This research underscores the necessity of understanding the mechanisms of RNA splicing dysregulation in order to develop targeted therapies to correct these aberrant processes, thereby improving outcomes for patients with leukemia and related disorders. Full article

► Show Figures

Figure 1

13 pages, 965 KiB

Open AccessArticle

Real-World Study of US Adults with Paroxysmal Nocturnal Hemoglobinuria Treated with Pegcetacoplan

by Brian Mulherin, Apeksha Shenoy, Lily Arnett, Weiqi Jiao, Joseph Guarinoni, Sujata Sarda, Jinny Min and David Dingli

Hematol. Rep. 2024, 16(4), 669-681; https://doi.org/10.3390/hematolrep16040065 - 29 Oct 2024

Abstract

Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening disease characterized by complement-mediated hemolysis. OPERA is the first US longitudinal real-world study on C3 inhibitor therapy, known as pegcetacoplan. Methods: OPERA enrolled US patients with PNH, age ≥18, who were [...] Read more.

Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening disease characterized by complement-mediated hemolysis. OPERA is the first US longitudinal real-world study on C3 inhibitor therapy, known as pegcetacoplan. Methods: OPERA enrolled US patients with PNH, age ≥18, who were prescribed pegcetacoplan, and data were collected from routine care. Hemoglobin was reported by patients during regular follow-up (censored from transfusions). The Functional Assessment of Chronic Illness Therapy (FACIT)-Fatigue (0–52 score) and Patient-Reported Outcomes Measurement Information System scale for Cognitive Function Abilities (PROMIS-CF; 23.27–67.09 t-score) were completed electronically (low score = negative outcome). Patients self-reported incidence of healthcare resource utilization (HCRU). Results: By January 2024, 70 patients (mean age 44.6 years; 57.1% female) reported up to 9 months of pegcetacoplan treatment, with a median [IQR] follow-up of 6.6 [3.8] months. The latest reported hemoglobin levels improved by a mean (SD) of 2.6 (1.9) g/dL from baseline. At 3, 6 and 9 months, patients reported clinically meaningful improvements (≥5 points) in FACIT-F (53.3–69.0%) and (≥2 points) PROMIS-CF (46.7–55.2%). Patients reported a <10% incidence rate per person month of all HCRU events. Conclusions: This first longitudinal real-world US study indicates a positive trend in Hb, fatigue, and cognition with limited HCRU during pegcetacoplan treatment in adults with PNH. Full article

► Show Figures

Figure 1

13 pages, 501 KiB

Open AccessReview

Gene Therapy: A Revolutionary Step in Treating Thalassemia

by Jhancy Malay, Rasha Aziz Attia Salama, Ghania Shehzad Alam Qureshi, Ali Raafat Ali Ahmed Ammar, Gayatri Janardhan, Maryam Safdar and Hesham Amin Hamdy Elshamy

Hematol. Rep. 2024, 16(4), 656-668; https://doi.org/10.3390/hematolrep16040064 - 21 Oct 2024

Abstract

Beta thalassemia is an inherited blood disorder that results in inefficient erythropoiesis due to genetic mutation that leads to the reduction or absence of the hemoglobin beta-globulin protein. Approximately 8.5% of UAE residents suffer from β-thalassemia, a significant health and financial problem. The [...] Read more.

Beta thalassemia is an inherited blood disorder that results in inefficient erythropoiesis due to genetic mutation that leads to the reduction or absence of the hemoglobin beta-globulin protein. Approximately 8.5% of UAE residents suffer from β-thalassemia, a significant health and financial problem. The treatment options available for β-Thalassemia major are limited and associated with a wide range of complications. β-thalassemia gene therapy is emerging as a potential novel treatment option that eliminates the complications caused by the current long-term treatment modalities and the associated economic burden. This paper reviews the scientific literature related to emerging gene therapy for β-Thalassemia by analyzing all the articles published from January 2010 to December 2023 in the English language on Databases like PubMed, Scopus, ProQuest, and CINAHL. The use of gene therapy has demonstrated promising outcomes for a permanent cure of β-Thalassemia. To conclude, gene therapy is an innovative solution. It demonstrates a promising future, but does come with its own setbacks and is something that must be tackled in order to revolutionize it in the medical world. FDA-approved ZYNTEGLO is a potentially one-time curative treatment for β-Thalassemia. Although cutting-edge, its use is limited because of the high cost—a price of USD 2.8 million per patient. Full article

► Show Figures

Figure 1

8 pages, 8489 KiB

Open AccessCase Report

Pancytopenia Related to Splenic Angiosarcoma: A Case Report and Literature Review

by Jakub Misiak, Bernard Sokołowski, Norbert Skrobisz, Mateusz Matczak and Marcin Braun

Hematol. Rep. 2024, 16(4), 648-655; https://doi.org/10.3390/hematolrep16040063 - 18 Oct 2024

Abstract

Background: Angiosarcomas are highly aggressive malignancies with endothelial differentiation, presenting considerable challenges in oncology, especially when arising in rare locations such as the spleen. These tumors predominantly affect adults and are commonly found in the skin, breast, liver, or soft tissues, with more [...] Read more.

Background: Angiosarcomas are highly aggressive malignancies with endothelial differentiation, presenting considerable challenges in oncology, especially when arising in rare locations such as the spleen. These tumors predominantly affect adults and are commonly found in the skin, breast, liver, or soft tissues, with more unusual occurrences in other organs. Angiosarcomas have a high propensity for metastasis, typically spreading to the liver, lungs, lymph nodes, and gastrointestinal tract. Splenic angiosarcoma, with fewer than 300 documented cases, is an especially rare and complex form of this malignancy. Case presentation: This report details a case of splenic angiosarcoma in a 45-year-old male, where bone marrow metastases were the first clinical presentation, initially mimicking myelodysplastic syndrome (MDS) due to persistent pancytopenia. Conclusions: The eventual identification of the splenic origin underscores the diagnostic difficulties and clinical challenges inherent in managing such atypical and rare presentations. Full article

► Show Figures

Figure 1

12 pages, 421 KiB

Open AccessArticle

Outcome of Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Acute Lymphoblastic Leukemia: Results of a Single-Center Study

by Davide Stella, Jessica Gill, Roberto Passera, Sofia Zompi, Chiara Maria Dellacasa, Ernesta Audisio, Marco Cerrano, Irene Dogliotti, Michele Dicataldo, Carolina Secreto, Benedetto Bruno, Roberto Freilone, Alessandro Busca and Luisa Giaccone

Hematol. Rep. 2024, 16(4), 636-647; https://doi.org/10.3390/hematolrep16040062 - 17 Oct 2024

Abstract

Background: Despite the adoption of pediatric-like chemotherapy protocols, the introduction of new immunotherapies and a better understanding of the oncogenic landscape, the outcome for adult patients with acute lymphoblastic leukemia (ALL) remain substantially dismal. The aim of the present study was to evaluate [...] Read more.

Background: Despite the adoption of pediatric-like chemotherapy protocols, the introduction of new immunotherapies and a better understanding of the oncogenic landscape, the outcome for adult patients with acute lymphoblastic leukemia (ALL) remain substantially dismal. The aim of the present study was to evaluate the outcome in terms of survival in a cohort of adult patients with ALL who received allogeneic hematopoietic stem cell transplantation (alloSCT) between 2013 and 2023. Methods: This was a single-center observational retrospective study including all consecutive adult patients with ALL who received an alloSCT between April 2013 and April 2023 at the Stem Cell Transplant Center AOU Città della Salute e della Scienza of Torino. The primary endpoints were overall survival (OS), graft-versus-host disease (GVHD) Relapse-Free Survival (GRFS), Leukemia-Free Survival (LFS) and cumulative incidence (CI) of Non-Relapse Mortality (NRM). Results: The 4-year OS and LFS were 63.4% and 48.1%, respectively, and the 1-year GRFS was 42.9%. The 1-year CI of bloodstream infections (BSI), invasive fungal infections and NRM were 38%, 7% and 18.4%, respectively. Multivariate analysis showed that the use of total body irradiation (TBI), a time interval from diagnosis to alloSCT less than 7 months and female gender were factors significantly associated with better OS. Relapse of the underlying malignancy and BSI were the main causes of death. Conclusion: Our study suggests that alloSCT from a matched sibling donor (MSD) and alternative donors may be considered an effective tool for patients with ALL achieving a CR. Full article

► Show Figures

Figure 1

12 pages, 1815 KiB

Open AccessArticle

Trabecular Attenuation of L1 in Adult Patients with Multiple Myeloma: An Observational Study on Low-Dose CT Images

by Carlo Augusto Mallio, Valeria Tomarchio, Francesco Pulcini, Edoardo Verducci, Caterina Bernetti, Maria Antonietta Tafuri, Federico Greco, Luigi Rigacci, Bruno Beomonte Zobel and Ombretta Annibali

Hematol. Rep. 2024, 16(4), 624-635; https://doi.org/10.3390/hematolrep16040061 - 17 Oct 2024

Abstract

Background: The aim of this study was to evaluate the impact of trabecular attenuation of the L1 vertebral body in low-dose CT in adult patients with multiple myeloma (MM), smoldering multiple myeloma (SMM), and monoclonal gammopathy of undetermined significance (MGUS). Materials and Methods: [...] Read more.

Background: The aim of this study was to evaluate the impact of trabecular attenuation of the L1 vertebral body in low-dose CT in adult patients with multiple myeloma (MM), smoldering multiple myeloma (SMM), and monoclonal gammopathy of undetermined significance (MGUS). Materials and Methods: The study population consisted of 22 patients with MGUS and 51 consecutive patients with newly diagnosed MM (SMM, n = 21; symptomatic MM, n = 36). CT scans were conducted using a 128-slice CT scanner (Somatom go.Top, Siemens, Munich, Germany). Low-dose whole-body CT scans were performed at a single time point for each patient. Trabecular bone density values were obtained by defining regions of interest on non-contrast images at the level of L1 vertebra. A threshold of p = 0.05 was applied to determine statistical significance. Results: The median Hounsfield unit (HU) value in patients with MGUS, SMM, and MM was 148 HU (range 81–190), 130 HU (range 93–193), and 92 HU (range 26–190), respectively, with a statistically significant difference between the groups (p = 0.0015). Patients with HU values ≤ 92 had lower progression-free survival with statistically significant differences compared to the group with HU values > 92 (p < 0.0499). Conclusions: This is the earliest evidence of the importance of evaluating L1 attenuation values in low-dose CT images in patients with MGUS, SMM, and MM. Further prospective studies could contribute to reinforcing these results and exploring the clinical applicability and generalization of L1 attenuation values in low-dose whole-body CT scans in routine clinical practice. Full article

► Show Figures

Figure 1

12 pages, 1795 KiB

Open AccessEditor’s ChoiceArticle

Utilizing Clinical Transformation Criteria for Prognostic Stratification in Follicular Lymphoma Prior to Initial Immunochemotherapy

by Yoshikazu Hori, Hiroki Hosoi, Takayuki Hiroi, Ke Wan, Shogo Murata, Masaya Morimoto, Toshiki Mushino, Akinori Nishikawa and Takashi Sonoki

Hematol. Rep. 2024, 16(4), 612-623; https://doi.org/10.3390/hematolrep16040060 - 4 Oct 2024

Abstract

Background: Although the prognosis of follicular lymphoma (FL) has improved, some patients experience early disease progression, including progression of disease within 24 months (POD24). Histological transformation is a critical event in FL. However, the heterogeneity of FL tumors makes it challenging to diagnose [...] Read more.

Background: Although the prognosis of follicular lymphoma (FL) has improved, some patients experience early disease progression, including progression of disease within 24 months (POD24). Histological transformation is a critical event in FL. However, the heterogeneity of FL tumors makes it challenging to diagnose transformation accurately. We retrospectively applied the clinical transformation criteria used for FL transformation assessments at relapse or disease progression to conduct transformation assessments before the initial immunochemotherapy. Methods: Sixty-six FL patients who first received immunochemotherapy between January 2009 and February 2023 at our institution were selected. Twenty-three were clinical-transformation-positive (CLT+). Results: The progression-free survival (PFS) rate of the CLT+ patients was significantly lower than that of the clinical-transformation-negative (CLT−) patients. In the POD24 assessment subgroup, the CLT+ patients had a higher incidence of POD24 than the CLT− patients. There was no significant difference in PFS between the patients treated with CHOP-like regimens and those treated with bendamustine regimens. In the CHOP-like group, the CLT+ patients exhibited significantly lower PFS than the CLT− patients. In the bendamustine group, the clinical transformation did not affect PFS. Conclusion: Clinical transformation criteria may be useful for the prognostic stratification of FL prior to immunochemotherapy. Additionally, they may serve as predictors of POD24. Full article

► Show Figures

Figure 1

9 pages, 3261 KiB

Open AccessEditor’s ChoiceArticle

Comprehensive Genomic Profiling in Non-Myeloid Hematologic Malignancies Identifies Variants That Can Alter Clinical Practice

by Chenyu Lin, Katherine I. Zhou, Michelle F. Green, Bennett A. Caughey, John H. Strickler, Michael B. Datto and Matthew S. McKinney

Hematol. Rep. 2024, 16(4), 603-611; https://doi.org/10.3390/hematolrep16040059 - 30 Sep 2024

Abstract

Background: Comprehensive genomic profiling (CGP) is frequently adopted to direct the clinical care of myeloid neoplasms and solid tumors, but its utility in the care of lymphoid and histiocytic cancers is less well defined. Methods: In this study, we aimed to evaluate the [...] Read more.

Background: Comprehensive genomic profiling (CGP) is frequently adopted to direct the clinical care of myeloid neoplasms and solid tumors, but its utility in the care of lymphoid and histiocytic cancers is less well defined. Methods: In this study, we aimed to evaluate the frequency at which mutations identified by CGP altered management in non-myeloid hematologic malignancies. We retrospectively examined the CGP results of 105 samples from 101 patients with non-myeloid hematologic malignancies treated at an academic medical center who had CGP testing between 2014 and 2021. Results: CGP revealed one or more pathogenic or likely pathogenic variant in 92 (88%) of samples and 73 (72%) of tested patients had one or more mutations with diagnostic, prognostic, or therapeutic significance. The identification of a resistance variant resulted in the suspension of the active treatment or affected subsequent treatment choice in 9 (69%) out of 13 patients. However, the presence of a therapy sensitizing variant only led to consideration of a biomarker-directed therapy in 6 (10%) out of 61 patients. Conclusions: Overall, CGP of non-myeloid hematologic malignancies identified clinically significant variants in 72% of patients and resulted in a change in management in 22% of patients. Full article

► Show Figures

Figure 1

Figure 1
Clinician-stated justification for sending CGP. The number of patients falling into each category is depicted in parentheses in the legend. In some cases (n = 7), clinicians cited multiple reasons for sending CGP. Full article ">Figure 2
Number (table, (left)) and proportion (graph, (right)) of patients who had therapy sensitizing variants identified by CGP (actionable) and in whom these variants led to a biomarker-directed therapy being offered (acted on), color coded by level of evidence. Full article ">Figure 3
Number (table, (left)) and proportion (graph, (right)) of patients who had variants conferring therapy resistance identified by CGP (actionable) and in whom these variants resulted in suspension of the active treatment or affected subsequent treatment choice (acted on), color coded by level of evidence. Full article ">Figure 4
Number (table, (left)) and proportion (graph, (right)) of patients who had variants of diagnostic significance identified by CGP (actionable) and in whom these variants were used to clarify a diagnosis (acted on), color coded by level of evidence. Full article ">Figure 5
Number (table, (left)) and proportion (graph, (right)) of patients who had variants of prognostic significance identified by CGP (actionable) and in whom these variants led to change sin clinical practice (acted on), color coded by level of evidence. Full article ">

10 pages, 999 KiB

Open AccessEditor’s ChoiceArticle

The Real-World Outcomes of Relapsed/Refractory Multiple Myeloma Treated with Elotuzumab, Pomalidomide, and Dexamethasone

by Hitomi Nakayama, Yoshinobu Aisa, Chisako Ito, Aki Sakurai and Tomonori Nakazato

Hematol. Rep. 2024, 16(4), 593-602; https://doi.org/10.3390/hematolrep16040058 - 30 Sep 2024

Abstract

Introduction: A combination of elotuzumab, pomalidomide, and dexamethasone (EPd) was approved for the treatment of relapsed/refractory multiple myeloma (RRMM) following the ELOQUENT-3 phase II clinical trial. However, the clinical experience with this therapy is still limited. In this retrospective study, we analyzed [...] Read more.

Introduction: A combination of elotuzumab, pomalidomide, and dexamethasone (EPd) was approved for the treatment of relapsed/refractory multiple myeloma (RRMM) following the ELOQUENT-3 phase II clinical trial. However, the clinical experience with this therapy is still limited. In this retrospective study, we analyzed the efficacy and safety of EPd in a real-world cohort of RRMM patients. Patients and Methods: The medical records of 22 patients who received EPd for RRMM at Yokohama Municipal Citizen’s Hospital (Japan) between January 2020 and July 2021 were reviewed. Results: The median age of our cohort was 73.5 years. The overall response rate was 55%. With a median follow-up of 20.2 months, the median progression-free survival (PFS) was 9.1 months (95% confidence interval [CI], 2.5–23.0 months). The median PFS was shorter in patients with a poor performance status (PS) than in those with favorable PS (2.5 vs. 10.8 months; p < 0.01). Patients with prior daratumumab had significantly shorter PFS than those without prior daratumumab (2.1 vs. 23.0 months; p < 0.01). Additionally, patients with prior pomalidomide had significantly shorter PFS (1.7 vs. 10.3 months; p < 0.01). In the multivariate analysis, poor PS (hazard ratio [HR] = 4.1, 95% CI: 1.1–15.6; p = 0.04) and prior exposure to daratumumab (HR = 3.8, 95% CI: 1.1–13.8; p = 0.04) remained significantly associated with shorter PFS. Conclusions: The results of our study suggest that EPd is an active and well-tolerated regimen in RRMM, even in real-world patients. Furthermore, EPd may be useful, especially in daratumumab-naïve patients. Full article

► Show Figures

Figure 1

8 pages, 874 KiB

Open AccessCase Report

Two Cases of Immune Thrombocytopenia (ITP) Related to Viral Vector Vaccination ChAdOx1-S (AstraZeneca) and a Good Response after Thrombopoietin Receptor Agonist (TPO-RA) Therapy

by Konstantina Salveridou, Theodoros Tzamalis, Maika Klaiber-Hakimi, Sabine Haase, Stefanie Gröpper and Aristoteles Giagounidis

Hematol. Rep. 2024, 16(4), 585-592; https://doi.org/10.3390/hematolrep16040057 - 27 Sep 2024

Abstract

Background: In 2019, a new coronavirus disease emerged in Wuhan, China, known as SARS-CoV-2, severe acute respiratory syndrome coronavirus 2, and caused an ongoing pandemic. Symptomatology of the syndrome is variable, with complications extending to hematopoiesis and hemostasis. Approximately a year after onset [...] Read more.

Background: In 2019, a new coronavirus disease emerged in Wuhan, China, known as SARS-CoV-2, severe acute respiratory syndrome coronavirus 2, and caused an ongoing pandemic. Symptomatology of the syndrome is variable, with complications extending to hematopoiesis and hemostasis. Approximately a year after onset of the virus, four vaccination formulas became available to the public, based on a viral vector or mRNA technology. These vaccine formulas have been hampered with hematological complications, like vaccine-induced immune thrombotic thrombocytopenia (VITT) and vaccine-related ITP (immune thrombocytopenic purpura). ITP is a disease with autoimmune pathogenesis characterized by antibody production against platelets and an increased hemorrhagic risk. A decent number of cases have been referred to as possible adverse effects of COVID-19 vaccinations. Case presentation: in this case report, we present two cases of newly diagnosed ITP after vaccination with ChAdOx1-S (AstraZeneca), with a good response to treatment with thrombopoietin-receptor agonists (TPO-RAs). Discussion: we observed an absence of response after corticosteroids and IVIG therapy and a positive therapeutic outcome on TPO-RA. Conclusions: in the ongoing pandemic, there is an urgent need to create therapeutic guidelines for vaccination-related clinical entities and to clarify indications for the vaccination of patients with pre-existing hematological diseases. Full article

► Show Figures

Figure 1

6 pages, 549 KiB

Open AccessCase Report

Complete Remission with Inotuzumab Ozogamicin as Fourth-Line Salvage Therapy in a Child with Relapsed/Refractory Acute Lymphoblastic Leukemia

by Athanasios Tragiannidis, Vassiliki Antari, Eleni Tsotridou, Theodoros Sidiropoulos, Aikaterini Kaisari, Maria Palabougiouki, Timoleon-Achilleas Vyzantiadis, Emmanuel Hatzipantelis, Assimina Galli-Tsinopoulou and Evgenios Goussetis

Hematol. Rep. 2024, 16(4), 579-584; https://doi.org/10.3390/hematolrep16040056 - 27 Sep 2024

Abstract

Background: Despite the progress achieved regarding survival rates in childhood acute lymphoblastic leukemia (ALL), relapsed or refractory disease still poses a therapeutic challenge. Inotuzumab ozogamicin is a CD22-directed monoclonal antibody conjugated to calicheamicin, which has been approved by the Food and Drug Administration [...] Read more.

Background: Despite the progress achieved regarding survival rates in childhood acute lymphoblastic leukemia (ALL), relapsed or refractory disease still poses a therapeutic challenge. Inotuzumab ozogamicin is a CD22-directed monoclonal antibody conjugated to calicheamicin, which has been approved by the Food and Drug Administration for adults and pediatric patients 1 year and older with relapsed or refractory CD22-positive B-cell precursor acute lymphoblastic leukemia. Case presentation: Herein, we present the case of a 23-month-old girl with high-risk B-ALL who experienced very early isolated medullary relapse; following the failure of conventional chemotherapy according to the ALL-IC REL 2016 protocol, she went on to receive the bispecific T-cell engager (BiTE) blinatumomab and subsequently, due to refractory disease, the combination of fludarabine, cytarabine, and the proteasome inhibitor bortezomib without achieving remission. Given the high CD22 expression by the lymphoblasts, off-label use of inotuzumab ozogamicin (InO) was chosen and administrated in a 28-day cycle as a salvage treatment. The minimal residual disease (MRD) was 0.08% on day 28, and InO was continued, thus achieving MRD negativity; the patient successfully underwent an allogeneic stem cell transplantation from a matched family donor. Conclusions: Our case highlights the efficacy and safety of InO as a salvage treatment in the setting of relapsed B-ALL refractory not only to conventional chemotherapy but also to novel treatments, such as blinatumomab and bortezomib. Full article

► Show Figures

Figure 1

11 pages, 619 KiB

Open AccessEditor’s ChoiceArticle

Padua Prediction Score and Hospital-Acquired Proximal and Isolated Distal Deep Vein Thrombosis in Symptomatic Patients

by Michelangelo Sartori, Miriam Fiocca, Mario Soldati, Laura Borgese, Elisabetta Favaretto and Benilde Cosmi

Hematol. Rep. 2024, 16(4), 568-578; https://doi.org/10.3390/hematolrep16040055 - 25 Sep 2024

Abstract

Background: Hospital-acquired deep vein thrombosis (DVT) is an important cause of morbidity and mortality. Objectives: The purpose of this study was to evaluate the prevalence of proximal lower limb DVT and isolated distal DVT (IDDVT) and their relationship to the Padua Prediction Score [...] Read more.

Background: Hospital-acquired deep vein thrombosis (DVT) is an important cause of morbidity and mortality. Objectives: The purpose of this study was to evaluate the prevalence of proximal lower limb DVT and isolated distal DVT (IDDVT) and their relationship to the Padua Prediction Score (PPS) in acutely ill, hospitalized patients. Methods: In a single-center cross-sectional study, all inpatients from medical departments with suspected lower-extremity DVT were evaluated with whole-leg ultrasonography during 183 days from 2016 to 2017. Results: Among the 505 inpatients (age 78.0 ± 13.3, females 59.2%) from medical departments, 204 (40.2%) had PPS ≥ 4, but only 54.4% of them underwent pharmacological thrombo-prophylaxis. Whole-leg ultrasonography detected 47 proximal DVTs (9.3%) and 65 IDDVTs (12.8%). Proximal DVT prevalence was higher in patients with high PPS vs. those with low PPS (12.7% vs. 7.0% p = 0.029, respectively), whereas IDDVT prevalence was similar in patients with high and low PPS (14.7% vs. 11.6% p = 0.311, respectively). The area under the receiver operating curve (AUC) for the PPS was 0.62 ± 0.03 for all DVTs, 0.64 ± 0.04 for proximal DVTs, and 0.58 ± 0.04 for IDDVTs. Conclusions: In hospitalized patients, IDDVT had similar prevalence regardless of PPS risk stratification. Adherence to thrombo-prophylaxis in patients was still far from optimal. Full article

► Show Figures

Figure 1

Journal Description

Hematology Reports

Latest Articles

Journal Menu

Journal Browser

Highly Accessed Articles

Latest Books

E-Mail Alert

News

Topics

Conferences

Special Issues

Further Information

Guidelines

MDPI Initiatives

Follow MDPI