Haematological Neoplasms: Diagnosis and Management

A special issue of Current Oncology (ISSN 1718-7729). This special issue belongs to the section "Hematology".

Deadline for manuscript submissions: closed (30 September 2023) | Viewed by 37858

Special Issue Editors


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Guest Editor
Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, 05100 Terni, Italy
Interests: hematopathology; immunohistochemistry; lymphoid neoplasms; myeloid neoplasms

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Guest Editor
Department of Pathology, University of Foggia School of Medicine, 71121 Foggia, Italy
Interests: genitourinary pathology; diagnostic biomarkers; prognostic/predictive biomarkers; digital pathology; emolymphopathology; bladder cancer; prostate cancer
Special Issues, Collections and Topics in MDPI journals

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Guest Editor
Pathology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, Italy
Interests: surgical pathology; hematopathology; lymphomas; molecular pathology

Special Issue Information

Dear Colleagues,

The latest WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th Edition, dates back to 2017, and there is a huge ongoing work for the new edition to be published within the coming years as part of the novel 5th Edition series. This work is focused on the analysis, discussion, and potential endorsement of the recent advances in the pathology and molecular biology of haematological neoplasms.

We are pleased to invite you to submit your research works to this Special Issue, which aims to shape the current landscape of the diagnosis and management of haematological neoplasms of both myeloid and lymphoid origin by critically assessing the more recent technological advances in the recognition of novel pathological entities, the detection of prognostic and predictive biomarkers, and the application of precision medicine tools.

In this Special Issue, original research articles and reviews are welcome. Research areas may include (but are not limited to) ancillary techniques (including immunohistochemistry, PCR-based methods, and cytogenetics), the identification of prognostic/predictive biomarkers, issues in the diagnosis/differential diagnosis of challenging entities, and the current role of pathology in tailored therapy and precision medicine.

We look forward to receiving your contributions.

Prof. Dr. Stefano Ascani
Prof. Dr. Francesca Sanguedolce
Dr. Magda Zanelli
Guest Editors

Manuscript Submission Information

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Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2200 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • myeloid neoplasms
  • lymphoid neoplasms
  • pathology
  • molecular biology
  • precision medicine
  • biomarkers

Published Papers (18 papers)

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Research

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19 pages, 6713 KiB  
Article
A Novel Fatty Acid Metabolism-Associated Risk Model for Prognosis Prediction in Acute Myeloid Leukaemia
by Nana Wang, Xiaoran Bai, Xinlu Wang, Dongmei Wang, Guangxin Ma, Fan Zhang, Jingjing Ye, Fei Lu and Chunyan Ji
Curr. Oncol. 2023, 30(2), 2524-2542; https://doi.org/10.3390/curroncol30020193 - 20 Feb 2023
Cited by 1 | Viewed by 1887
Abstract
Acute myeloid leukaemia (AML) is the most common acute leukaemia in adults, with an unfavourable outcome and a high rate of recurrence due to its heterogeneity. Dysregulation of fatty acid metabolism plays a crucial role in the development of several tumours. However, the [...] Read more.
Acute myeloid leukaemia (AML) is the most common acute leukaemia in adults, with an unfavourable outcome and a high rate of recurrence due to its heterogeneity. Dysregulation of fatty acid metabolism plays a crucial role in the development of several tumours. However, the value of fatty acid metabolism (FAM) in the progression of AML remains unclear. In this study, we obtained RNA sequencing and corresponding clinicopathological information from the TCGA and GEO databases. Univariate Cox regression analysis and subsequent LASSO Cox regression analysis were utilized to identify prognostic FAM-related genes and develop a potential prognostic risk model. Kaplan-Meier analysis was used for prognostic significances. We also performed ROC curve to illustrate that the risk model in prognostic prediction has good performance. Moreover, significant differences in immune infiltration landscape were found between high-risk and low-risk groups using ESTIMATE and CIBERSOT algorithms. In the end, differential expressed genes (DEGs) were analyzed by gene set enrichment analysis (GSEA) to preliminarily explore the possible signaling pathways related to the prognosis of FAM and AML. The results of our study may provide potential prognostic biomarkers and therapeutic targets for AML patients, which is conducive to individualized precision therapy. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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7 pages, 443 KiB  
Communication
Survey of Anaphylaxis during Rasburicase Re-Administration in Patients with Hematological Malignancies Using a Japanese Claims Database
by Shunsuke Kobayashi, Takeo Yasu and Manabu Akazawa
Curr. Oncol. 2022, 29(12), 9826-9832; https://doi.org/10.3390/curroncol29120772 - 14 Dec 2022
Cited by 1 | Viewed by 1779
Abstract
Management of tumor lysis syndrome (TLS) associated with cancer chemotherapy for malignant tumors is important because of its potentially fatal course. The use of rasburicase, a recombinant urate oxidase, is recommended for TLS; however, because rasburicase is an enzymatic drug, one should be [...] Read more.
Management of tumor lysis syndrome (TLS) associated with cancer chemotherapy for malignant tumors is important because of its potentially fatal course. The use of rasburicase, a recombinant urate oxidase, is recommended for TLS; however, because rasburicase is an enzymatic drug, one should be cautious of anaphylaxis during administration. Using claims data in Japan, we investigated the rate of rasburicase re-administration and the occurrence of anaphylaxis during re-administration in patients with hematopoietic malignancies in a multicenter setting. Re-administration of rasburicase was defined as administration after an interval of 21 days from the first dose. Of 373 patients, 18 were re-administered rasburicase (re-administration rate: 4.8%). No patient developed anaphylaxis. The median number of days from the first to the last dose of rasburicase was 256.5 days (interquartile range: 138.8–455.8 days). The median daily dose was 7.5 mg (4.5–11.3 mg), and the median total dose was 33.8 mg (19.1–64.1 mg). This claims database analysis revealed that the re-administration rate of rasburicase was low in Japanese patients with hematopoietic malignancies, suggesting that rasburicase was being used appropriately, and that associated anaphylaxis was not observed. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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14 pages, 2364 KiB  
Article
Decreased FOXO1 Expression Is Correlated with Poor Prognosis in Myelodysplastic Syndromes
by Zheng Zhang, Nanfang Huang, Feng Xv, Sida Zhao, Juan Guo, Youshan Zhao and Chunkang Chang
Curr. Oncol. 2022, 29(10), 6933-6946; https://doi.org/10.3390/curroncol29100545 - 25 Sep 2022
Viewed by 1691
Abstract
Myelodysplastic syndrome is one of the main hematological malignancies that threaten the health of the elderly. However, biomarkers which predict the progression and prognosis of MDS are still controversial and puzzling. FOXO1 gene plays an important role in a variety of intracellular functions, [...] Read more.
Myelodysplastic syndrome is one of the main hematological malignancies that threaten the health of the elderly. However, biomarkers which predict the progression and prognosis of MDS are still controversial and puzzling. FOXO1 gene plays an important role in a variety of intracellular functions, including tumor suppression and cellular immune regulation. However, there is no research report on the correlation between FOXO1 and the clinical features of MDS including immune environment. In this study, we observed that FOXO1 expression is associated with neutrophil count, blasts, chromosome and different MDS scoring systems. FOXO1 expression is closely related to MDS cell immune polarization, and the increase expression of FOXO1 is significantly related to the amplification of immune cell polarization ratio. In addition, FOXO1 expression is associated with progression-free survival and overall survival in MDS patients. Moreover, in a multivariate model FOXO1 low-expression was an independent predictor of poor survival in MDS. In summary, FOXO1 may play a candidate tumor suppressor in MDS, and FOXO1 is a useful independent prognostic predictor in MDS, and it may provide a candidate target therapy in future. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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12 pages, 1894 KiB  
Article
The Down-Regulation of Circ_0059707 in Acute Myeloid Leukemia Promotes Cell Growth and Inhibits Apoptosis by Regulating miR-1287-5p
by Jichun Ma, Xiangmei Wen, Zijun Xu, Peihui Xia, Ye Jin, Jiang Lin and Jun Qian
Curr. Oncol. 2022, 29(9), 6688-6699; https://doi.org/10.3390/curroncol29090525 - 18 Sep 2022
Cited by 3 | Viewed by 2147
Abstract
Acute myeloid leukemia (AML) is the most common type of hematological malignancy. Recently, an increasing number of reports have shown that many circular RNAs can act as effective targets for AML. However, the roles of circ_0059707 in AML remain largely unclear. In this [...] Read more.
Acute myeloid leukemia (AML) is the most common type of hematological malignancy. Recently, an increasing number of reports have shown that many circular RNAs can act as effective targets for AML. However, the roles of circ_0059707 in AML remain largely unclear. In this study, we found that the expression levels of circ_0059707 were significantly decreased in AML patients with respect to normal controls (p < 0.001). Low expression levels of circ_0059707 were also associated with a poor prognosis. Furthermore, circ_0059707 overexpression inhibited cell growth and promoted apoptosis in leukemia cells, compared with control cells. Circ_0059707- and empty plasmid-transfected cells were injected subcutaneously into BALB/c nude mice. We found that the tumor volume was significantly lower in mice in the circ_0059707 group than in control mice (p < 0.01). Nuclear pyknosis, nuclear fragmentation, nuclear dissolution, and cell necrosis were observed in the circ_0059707 group by HE staining. CircInteractome analysis showed that 25 microRNAs (miRNAs), including miR-1287-5p, ©-miR-1825, a©hsa-miR-326, may be potential targets for circ_0059707. The expression of these miRNAs was analyzed in both the GEO GSE51908 and the GSE142700 databases. miR-1287-5p expression was lower in AML patients compared with controls in both the GSE51908 and the GSE142700 datasets. Moreover, we demonstrated that miR-1287-5p expression was down-regulated in AML patients and up-regulated in circ_0059707-overexpressing cells. Collectively, our research demonstrated that the down-regulation of circ_0059707 was highly evident in de novo AML patients. Our analysis also demonstrated that circ_0059707 inhibited cell growth and promoted apoptosis by up-regulating miR-1287-5p. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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18 pages, 8745 KiB  
Article
Cutaneous Involvement in Diseases with Plasma Cell Differentiation: Diagnostic Approach
by Magda Zanelli, Andrea Palicelli, Francesca Sanguedolce, Maurizio Zizzo, Alessandra Filosa, Linda Ricci, Camilla Cresta, Giovanni Martino, Alessandra Bisagni, Eleonora Zanetti, Francesco di Donato, Beatrice Melli, Alessandra Soriano, Luca Cimino, Alberto Cavazza, Lisa Francesca Vivian and Stefano Ascani
Curr. Oncol. 2022, 29(5), 3026-3043; https://doi.org/10.3390/curroncol29050246 - 24 Apr 2022
Cited by 2 | Viewed by 3470
Abstract
Neoplasms with plasma cell differentiation may occasionally involve the skin. Cutaneous lesions may represent the first sign of an underlying systemic plasma cell malignancy, such as multiple myeloma, or the skin itself may be the primary site of occurrence of a hematological tumor [...] Read more.
Neoplasms with plasma cell differentiation may occasionally involve the skin. Cutaneous lesions may represent the first sign of an underlying systemic plasma cell malignancy, such as multiple myeloma, or the skin itself may be the primary site of occurrence of a hematological tumor with plasma cell differentiation. Starting from examples encountered in our daily practice, we discussed the diagnostic approach pathologists and clinicians should use when faced with cutaneous lesions with plasma cell differentiation. Cases of primary cutaneous marginal zone lymphoma, localized primary amyloidosis/amyloidoma, and cutaneous manifestations (secondary either to multiple myeloma or to plasmablastic lymphoma) are discussed, focusing on the importance of the adequate patient’s work-up and precise clinicopathological correlation to get to the correct diagnosis and appropriate treatment. The pertinent literature has been reviewed, and the clinical presentation, pathological findings, main differential diagnoses, treatment, and outcome of neoplasms with plasma cell differentiation involving the skin are discussed. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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Review

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20 pages, 9434 KiB  
Review
Management of Patients with Lower-Risk Myelodysplastic Neoplasms (MDS)
by Josephine Lucero, Salman Al-Harbi and Karen W. L. Yee
Curr. Oncol. 2023, 30(7), 6177-6196; https://doi.org/10.3390/curroncol30070459 - 27 Jun 2023
Viewed by 1945
Abstract
Myelodysplastic neoplasms (MDS) are a heterogenous group of clonal hematologic disorders characterized by morphologic dysplasia, ineffective hematopoiesis, and cytopenia. In the past year, the classification of MDS has been updated in the 5th edition of the World Health Organization (WHO) Classification of Haematolymphoid [...] Read more.
Myelodysplastic neoplasms (MDS) are a heterogenous group of clonal hematologic disorders characterized by morphologic dysplasia, ineffective hematopoiesis, and cytopenia. In the past year, the classification of MDS has been updated in the 5th edition of the World Health Organization (WHO) Classification of Haematolymphoid Tumours and the International Consensus Classification (ICC) of Myeloid Neoplasms and Acute Leukemia with incorporation of morphologic, clinical, and genomic data. Furthermore, the more comprehensive International Prognostic Scoring System-Molecular (IPSS-M) allows for improved risk stratification and prognostication. These three developments allow for more tailored therapeutic decision-making in view of the expanding treatment options in MDS. For patients with lower risk MDS, treatment is aimed at improving cytopenias, usually anemia. The recent approval of luspatercept and decitabine/cedazuridine have added on to the current armamentarium of erythropoietic stimulating agents and lenalidomide (for MDS with isolated deletion 5q). Several newer agents are being evaluated in phase 3 clinical trials for this group of patients, such as imetelstat and oral azacitidine. This review provides a summary of the classification systems, the prognostic scores and clinical management of patients with lower risk MDS. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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13 pages, 831 KiB  
Review
Novel Tools for Diagnosis and Monitoring of AML
by Francesca Guijarro, Marta Garrote, Neus Villamor, Dolors Colomer, Jordi Esteve and Mónica López-Guerra
Curr. Oncol. 2023, 30(6), 5201-5213; https://doi.org/10.3390/curroncol30060395 - 23 May 2023
Cited by 4 | Viewed by 2352
Abstract
In recent years, major advances in the understanding of acute myeloid leukemia (AML) pathogenesis, together with technological progress, have led us into a new era in the diagnosis and follow-up of patients with AML. A combination of immunophenotyping, cytogenetic and molecular studies are [...] Read more.
In recent years, major advances in the understanding of acute myeloid leukemia (AML) pathogenesis, together with technological progress, have led us into a new era in the diagnosis and follow-up of patients with AML. A combination of immunophenotyping, cytogenetic and molecular studies are required for AML diagnosis, including the use of next-generation sequencing (NGS) gene panels to screen all genetic alterations with diagnostic, prognostic and/or therapeutic value. Regarding AML monitoring, multiparametric flow cytometry and quantitative PCR/RT-PCR are currently the most implemented methodologies for measurable residual disease (MRD) evaluation. Given the limitations of these techniques, there is an urgent need to incorporate new tools for MRD monitoring, such as NGS and digital PCR. This review aims to provide an overview of the different technologies used for AML diagnosis and MRD monitoring and to highlight the limitations and challenges of current versus emerging tools. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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16 pages, 2808 KiB  
Review
Musculoskeletal Chronic Graft versus Host Disease—A Rare Complication to Allogeneic Hematopoietic Stem Cell Transplant: A Case-Based Report and Review of the Literature
by Alexander Dåtland Kvinge, Tobias Kvammen, Hrvoje Miletic, Laurence Albert Bindoff and Håkon Reikvam
Curr. Oncol. 2022, 29(11), 8415-8430; https://doi.org/10.3390/curroncol29110663 - 03 Nov 2022
Cited by 1 | Viewed by 1697
Abstract
Musculoskeletal graft versus host disease (GVHD) is a rare manifestation of chronic GVHD (cGVHD) following allogeneic hematopoietic stem cell transplantation (allo-HSCT). Left untreated, the disease can cause extensive damage to muscle tissue and joints. We describe a 62-year-old male with musculoskeletal GVHD and [...] Read more.
Musculoskeletal graft versus host disease (GVHD) is a rare manifestation of chronic GVHD (cGVHD) following allogeneic hematopoietic stem cell transplantation (allo-HSCT). Left untreated, the disease can cause extensive damage to muscle tissue and joints. We describe a 62-year-old male with musculoskeletal GVHD and generalized muscle pain and stiffness. In addition, we performed a systemic literature review based on published cases of musculoskeletal GVHD between 1983 and 2019. We identified 85 cases, 62% male and 38% female with an age of 4–69 years and median age of 39 years at diagnosis. The majority of patients (72%) also had manifestations of cGVHD in at least one other organ system, most frequently the skin (52%), followed by oropharyngeal mucosa (37%), and pulmonary and gastrointestinal tract (GI tract) (21%). We conclude that, while musculoskeletal cGVHD is a rare complication of allo-HSCT, it remains a serious and debilitating risk that must be considered in patients with muscle pain, muscle weakness, joint stiffness, and tissue inflammation. Early intervention is critical for the patient’s prognosis. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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14 pages, 4959 KiB  
Review
Primary Vitreoretinal Lymphoma: Current Diagnostic Laboratory Tests and New Emerging Molecular Tools
by Beatrice Melli, Pietro Gentile, Davide Nicoli, Enrico Farnetti, Stefania Croci, Fabrizio Gozzi, Elena Bolletta, Luca De Simone, Francesca Sanguedolce, Andrea Palicelli, Maurizio Zizzo, Stefano Ricci, Fiorella Ilariucci, Cristiana Rossi, Alberto Cavazza, Stefano Ascani, Luca Cimino and Magda Zanelli
Curr. Oncol. 2022, 29(10), 6908-6921; https://doi.org/10.3390/curroncol29100543 - 24 Sep 2022
Cited by 4 | Viewed by 2296
Abstract
Primary vitreoretinal lymphoma (PVRL), a rare aggressive malignancy primarily involving the retina and/or the vitreous, is a major diagnostic challenge for clinicians (who commonly misdiagnose it as chronic uveitis) as well as for pathologists (for biological and technical reasons). Delays in diagnosis and [...] Read more.
Primary vitreoretinal lymphoma (PVRL), a rare aggressive malignancy primarily involving the retina and/or the vitreous, is a major diagnostic challenge for clinicians (who commonly misdiagnose it as chronic uveitis) as well as for pathologists (for biological and technical reasons). Delays in diagnosis and treatment are responsible for visual impairments and life-threatening consequences, usually related to central nervous system involvement. The identification of lymphoma cells in vitreous fluid, obtained by vitrectomy, is required for diagnosis. Of note, the scarcity of neoplastic cells in small volumes of vitreous sample, and the fragility of lymphoma cells with degenerative changes caused by previous steroid use for presumed uveitis makes diagnosis based on cytology plus immunophenotyping difficult. Interleukin levels, immunoglobulin heavy chain or T-cell receptor gene rearrangements, and MYD88 mutation are applied in combination with cytology to support diagnosis. We aim to describe the current laboratory technologies for PVRL diagnosis, focusing on the main issues that these methods have. In addition, new emerging diagnostic strategies, such as next-generation sequencing analysis, are discussed. The genetic profile of PVRL remains largely unexplored. Better knowledge of genetic alterations is critical for precision medicine interventions with target-based treatments of this lymphoma for which no standardised treatment protocol currently exists. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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13 pages, 3858 KiB  
Review
Primary Diffuse Large B-Cell Lymphoma of the Urinary Bladder: Update on a Rare Disease and Potential Diagnostic Pitfalls
by Magda Zanelli, Francesca Sanguedolce, Maurizio Zizzo, Andrea Palicelli, David Pellegrini, Sabrina Farinacci, Alessandra Soriano, Elisabetta Froio, Luigi Cormio, Giuseppe Carrieri, Alberto Cavazza, Francesco Merli, Stefano A. Pileri and Stefano Ascani
Curr. Oncol. 2022, 29(2), 956-968; https://doi.org/10.3390/curroncol29020081 - 10 Feb 2022
Cited by 3 | Viewed by 2728
Abstract
Diffuse large B-cell lymphoma (DLBCL) represents the most frequent type of non-Hodgkin lymphoma. Globally, DLBCL is an aggressive disease, requiring an accurate diagnosis and prompt treatment. The diagnosis is often made on biopsy samples of a nodal mass, however, approximately 40% of DLBCL [...] Read more.
Diffuse large B-cell lymphoma (DLBCL) represents the most frequent type of non-Hodgkin lymphoma. Globally, DLBCL is an aggressive disease, requiring an accurate diagnosis and prompt treatment. The diagnosis is often made on biopsy samples of a nodal mass, however, approximately 40% of DLBCL cases arise at extranodal sites. The most common extranodal site is the gastrointestinal tract, however any extranodal area may be primarily involved. Primary urinary bladder lymphoma represents only 0.2% of extranodal non-Hodgkin lymphomas, whereas secondary involvement of the urinary bladder by a systemic lymphoma is a more common event. Despite being rare, DLBCL is considered to represent the predominant primary urinary bladder lymphoma. The majority of cases reported in the bladder belong to the DLBCL, NOS group, and there are only rare cases of EBV-positive DLBCL, NOS. In this review, we summarize the current knowledge on DLBCL primarily occurring in the urinary bladder, with the aim of increasing clinician and pathologist awareness on this aggressive lymphoma rarely arising in the urinary bladder. Additionally, we focus on those entities which should be taken into consideration in the differential diagnosis, highlighting potential diagnostic pitfalls. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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Other

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12 pages, 6936 KiB  
Case Report
Long-Smoldering T-prolymphocytic Leukemia: A Case Report and a Review of the Literature
by Hilde K. Gjelberg, Lars Helgeland, Knut Liseth, Francesca Micci, Miriam Sandnes, Hege G. Russnes and Håkon Reikvam
Curr. Oncol. 2023, 30(11), 10007-10018; https://doi.org/10.3390/curroncol30110727 - 18 Nov 2023
Viewed by 1391
Abstract
T-prolymphocytic leukemia (T-PLL) is a rare malignancy of mature T-cells with distinct clinical, cytomorphological, and molecular genetic features. The disease typically presents at an advanced stage, with marked leukocytosis, B symptoms, hepatosplenomegaly, and bone marrow failure. It usually follows an aggressive course from [...] Read more.
T-prolymphocytic leukemia (T-PLL) is a rare malignancy of mature T-cells with distinct clinical, cytomorphological, and molecular genetic features. The disease typically presents at an advanced stage, with marked leukocytosis, B symptoms, hepatosplenomegaly, and bone marrow failure. It usually follows an aggressive course from presentation, and the prognosis is often considered dismal; the median overall survival is less than one year with conventional chemotherapy. This case report describes a patient with T-PLL who, after an unusually protracted inactive phase, ultimately progressed to a highly invasive, organ-involving disease. After initial treatments failed, a novel treatment approach resulted in a significant response. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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24 pages, 386 KiB  
Systematic Review
Multiple Myeloma in 2023 Ways: From Trials to Real Life
by Manlio Fazio, Vittorio Del Fabro, Nunziatina Laura Parrinello, Alessandro Allegra, Uroš Markovic, Cirino Botta, Fabrizio Accardi, Iolanda Donatella Vincelli, Salvatore Leotta, Federica Elia, Benedetta Esposito, Bruno Garibaldi, Gabriele Sapuppo, Alessandra Orofino, Alessandra Romano, Giuseppe A. Palumbo, Francesco Di Raimondo and Concetta Conticello
Curr. Oncol. 2023, 30(11), 9710-9733; https://doi.org/10.3390/curroncol30110705 - 03 Nov 2023
Cited by 1 | Viewed by 2413
Abstract
Multiple myeloma is a chronic hematologic malignancy that obstinately tends to relapse. Basic research has made giant strides in better characterizing the molecular mechanisms of the disease. The results have led to the manufacturing of new, revolutionary drugs which have been widely tested [...] Read more.
Multiple myeloma is a chronic hematologic malignancy that obstinately tends to relapse. Basic research has made giant strides in better characterizing the molecular mechanisms of the disease. The results have led to the manufacturing of new, revolutionary drugs which have been widely tested in clinical trials. These drugs have been approved and are now part of the therapeutic armamentarium. As a consequence, it is essential to combine what we know from clinical trials with real-world data in order to improve therapeutic strategies. Starting with this premise, our review aims to describe the currently employed regimens in multiple myeloma and compare clinical trials with real-life experiences. We also intend to put a spotlight on promising therapies such as T-cell engagers and chimeric antigen receptor T-cells (CAR-T) which are proving to be effective in changing the course of advanced-stage disease. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
11 pages, 3863 KiB  
Case Report
Severe Fatal Mucormycosis in a Patient with Chronic Lymphocytic Leukaemia Treated with Zanubrutinib: A Case Report and Review of the Literature
by Giuseppe Maggioni, Marny Fedrigo, Andrea Visentin, Elisa Carturan, Valeria Ruocco, Livio Trentin, Mauro Alaibac and Annalisa Angelini
Curr. Oncol. 2023, 30(9), 8255-8265; https://doi.org/10.3390/curroncol30090599 - 07 Sep 2023
Cited by 1 | Viewed by 1284
Abstract
Severe mucormycosis is a fatal disease rarely complicating chronic lymphoproliferative disorders. We present a fulminant and fatal case of a 74-year-old Caucasian woman suffering from CLL treated with second-generation BTK inhibitor zanubrutinib. After a first septic episode a month prior, originating from the [...] Read more.
Severe mucormycosis is a fatal disease rarely complicating chronic lymphoproliferative disorders. We present a fulminant and fatal case of a 74-year-old Caucasian woman suffering from CLL treated with second-generation BTK inhibitor zanubrutinib. After a first septic episode a month prior, originating from the lung with later systemic involvement by an unidentified agent and treated with large-spectrum antibiotics and fluconazonle, a slow-onset enlarging tender warm and erythematous nodular swollen cutaneous lesion appeared in her lower limbs and spread subsequently to her upper limbs, progressing towards central ulceration with a necrotic core. Suspecting a mycotic dissemination from an unknown agent, a skin punch biopsy was performed, and intraconazole was started. Due to spread of the skin lesions, the patient was hospitalized and intravenous liposomal ampthotericin B was started. Histopathology showed an atypical sporangium-rich mycotic angioinvasion of the small vessels. Only the increase of BDG and GM could corroborate the hypothesis of mycotic infection. However, long-term CLL, immunosuppressive therapies, neutropenia, and prior use of azoles and other antimycotic agents were risk factors for mucormycosis; BTK inhibitor could also be added as another novel risk factor. Despite all therapeutic efforts, the patient died. Post-mortem molecular exams confirmed the diagnosis of disseminated mucormycosis. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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14 pages, 578 KiB  
Systematic Review
A Systematic Review Assessing the Impact of Vitamin D Levels on Adult Patients with Lymphoid Malignancies
by Cristina Potre, Ema Borsi, Ovidiu Potre, Ioana Ionita, Miruna Samfireag, Dan Costachescu, Cristina Secosan, Sandra Lazar and Anca Irina Ristescu
Curr. Oncol. 2023, 30(4), 4351-4364; https://doi.org/10.3390/curroncol30040331 - 20 Apr 2023
Cited by 4 | Viewed by 2199
Abstract
Vitamin D deficiency has been correlated with various conditions, including the risk of developing lymphoid malignancies. This systematic review aimed to assess the association between vitamin D levels at diagnosis of lymphoid malignancies, patient outcomes, and survival. A systematic review was conducted, encompassing [...] Read more.
Vitamin D deficiency has been correlated with various conditions, including the risk of developing lymphoid malignancies. This systematic review aimed to assess the association between vitamin D levels at diagnosis of lymphoid malignancies, patient outcomes, and survival. A systematic review was conducted, encompassing 15 studies published until January 2023, involving 4503 patients, examining the relationship between vitamin D and lymphoid cancers. The median age of the patients was 56.5 years, with a median follow-up duration of approximately 36 months across studies. The overall median vitamin D level at initial measurement was 20.4 ng/mL, while a <20 ng/mL threshold was used to define vitamin D insufficiency. The results demonstrated significant associations between vitamin D levels and patient outcomes in several lymphoid malignancies, with a pooled risk in disease progression of 1.93 and a pooled hazard ratio of 2.06 for overall survival in patients with 25-(OH)D levels below the normal threshold of 20 ng/mL. Among findings, it was demonstrated that supplemental vitamin D improves the chemosensitivity of tumors by reducing the rate of tumor growth compared with vitamin D or chemotherapy alone. Vitamin D had a protective effect for patients with DLBCL under R-CHOP treatment, while vitamin D insufficiency was associated with the impairment of rituximab treatment and showed worse clinical outcomes in chimeric antigen receptor T-cell (CAR-T) recipients. Although one study found no association between vitamin D deficiency and the cause of death, most associated vitamin D insufficiency with early clinical failure and lower survival probability. In conclusion, his systematic review highlights the importance of vitamin D levels in the prognosis and survival of patients with lymphoid malignancies. Further research is needed to better understand the underlying mechanisms and explore the potential benefits of vitamin D supplementation in managing these cancers. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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8 pages, 2603 KiB  
Case Report
CD5-Negative Primary Mantle Cell Lymphoma Presenting with a Bilateral Conjunctival Mass: A Potential Diagnostic Pitfall
by Magda Zanelli, Alberto Lugli, Andrea Palicelli, Francesca Sanguedolce, Maurizio Zizzo, Camilla Cresta, Samuele Biancafarina, Giovanni Martino, Barbara Crescenzi, Saverio Pancetti, Giuseppe Broggi, Rosario Caltabiano, Luca Cimino, Cristina Mecucci and Stefano Ascani
Curr. Oncol. 2023, 30(1), 824-831; https://doi.org/10.3390/curroncol30010062 - 06 Jan 2023
Viewed by 1486
Abstract
Mantle cell lymphoma is a B-cell malignancy, which, in its classic form, usually involves lymph nodes and extranodal sites, and, among the extranodal sites, the gastrointestinal tract and the Waldeyer’s ring are most prevalent. MCL is rarely reported in the ocular adnexa, a [...] Read more.
Mantle cell lymphoma is a B-cell malignancy, which, in its classic form, usually involves lymph nodes and extranodal sites, and, among the extranodal sites, the gastrointestinal tract and the Waldeyer’s ring are most prevalent. MCL is rarely reported in the ocular adnexa, a site more frequently affected by extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, which is a form of low-grade malignancy. The diagnosis of MCL presenting in the ocular adnexa requires special attention as its rarity in this location combined with the not uncommon CD5 negativity of the disease when occurring in the ocular adnexa, may lead the pathologist to overlook the diagnosis and misinterpret MCL as marginal zone B cell lymphoma, which has a totally different behavior. Herein, we present a case of primary bilateral conjunctival CD5-negative MCL in a patient having no other sites affected by lymphoma and we discuss possible diagnostic pitfalls. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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9 pages, 1025 KiB  
Case Report
Long-Term Molecular Remission after Treatment with Imatinib in a Chronic Myeloid Leukemia Patient with Extreme Thrombocytosis Harboring Rare e14a3 (b3a3) BCR::ABL1 Transcript: A Case Report
by Xupai Zhang, Haoping Sun, Yi Su and Hai Yi
Curr. Oncol. 2022, 29(11), 8171-8179; https://doi.org/10.3390/curroncol29110645 - 28 Oct 2022
Cited by 2 | Viewed by 1657
Abstract
An atypical BCR::ABL1 fusion gene transcript in chronic myeloid leukemia (CML) patients, even those with variant Philadelphia (Ph) chromosome translocation, is very rare. In the present study, we report a case of CML (41 years, female) with extreme thrombocytosis at onset, with the [...] Read more.
An atypical BCR::ABL1 fusion gene transcript in chronic myeloid leukemia (CML) patients, even those with variant Philadelphia (Ph) chromosome translocation, is very rare. In the present study, we report a case of CML (41 years, female) with extreme thrombocytosis at onset, with the variant Ph chromosome and rare e14a3 (b3a3) BCR::ABL1 transcript. The patient was prescribed imatinib as a first-line therapy and subsequently achieved complete hematologic remission within 2 months and major molecular response (MMR) within 3 months, and the transcript was undetectable within half a year. During up to nine years of follow-up, the quantification of this rare fusion gene was consistently negative with no BCR::ABL1 kinase domain mutations. Furthermore, we collected previously reported CML cases with the e14a3 (b3a3) transcript that indicated that the e14a3 (b3a3) transcripts appeared to have a larger number of thrombocytosis and variant Ph translocations than CML in general. This subgroup of CML might have better responses and outcomes to imatinib than patients with common transcripts. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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8 pages, 3417 KiB  
Case Report
Plasmacytic Pleural Effusion as a Major Presentation of Angioimmunoblastic T-Cell Lymphoma: A Case Report
by Borui Li, Lin Nong, Jianhua Zhang, Wensheng Wang, Qian Wang, Yang Zhang, Shaomin Ren and Mangju Wang
Curr. Oncol. 2022, 29(10), 7637-7644; https://doi.org/10.3390/curroncol29100603 - 13 Oct 2022
Viewed by 1592
Abstract
Angioimmunoblastic T-cell lymphoma is one of the peripheral T-cell lymphomas. Reactive plasma cells can occasionally be observed in AITL patients’ peripheral blood and bone marrow. Plasmacytic pleural effusion as the presentation of AITL has not been reported before. The mechanisms of plasmacytic pleural [...] Read more.
Angioimmunoblastic T-cell lymphoma is one of the peripheral T-cell lymphomas. Reactive plasma cells can occasionally be observed in AITL patients’ peripheral blood and bone marrow. Plasmacytic pleural effusion as the presentation of AITL has not been reported before. The mechanisms of plasmacytic pleural effusion are not fully understood. Here we present an 82-year-old male with exuberant plasma cells in his pleural effusion in addition to his peripheral blood and bone marrow aspiration. By presenting this case, we would like to expand the spectrum of disease presentations in AITL and discuss the significance of flow cytometry in the differential diagnosis of pleural effusion. To our knowledge, this is the first case report in the literature, which will be crucial to assist the hematopathologist in accurate diagnosis and treatment. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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10 pages, 9450 KiB  
Brief Report
Diffuse Large B Cell Lymphoma Arising in Patients with Preexisting Hodgkin Lymphoma
by Emilio Bellitti, Pierluigi Masciopinto, Pellegrino Musto, Elena Arcuti, Luca Mastracci, Giuseppina Opinto, Sabino Ciavarella, Attilio Guarini, Gerardo Cazzato, Giorgina Specchia, Eugenio Maiorano, Francesco Gaudio and Giuseppe Ingravallo
Curr. Oncol. 2022, 29(9), 6115-6124; https://doi.org/10.3390/curroncol29090480 - 25 Aug 2022
Cited by 1 | Viewed by 1996
Abstract
The metachronic onset of diffuse large B-cell lymphoma (DLBCL) after classic Hodgkin lymphoma (cHL) is a rare event affecting patients’ outcomes. However, although several studies have investigated the prognostic role of this event, little is known about a hypothetical common origin of the [...] Read more.
The metachronic onset of diffuse large B-cell lymphoma (DLBCL) after classic Hodgkin lymphoma (cHL) is a rare event affecting patients’ outcomes. However, although several studies have investigated the prognostic role of this event, little is known about a hypothetical common origin of the two different neoplastic cells. Aims: To investigate a possible relationship between DLBCL and cHL, in this retrospective study of 269 patients with newly diagnosed cHL treated at Bari University Hospital (Italy) between 2007 and 2020, we analyzed data from 4 patients (3 male and 1 female) with cHL who subsequently developed DLBCL. Methods: Gene expression profile analysis, assessed by NanoString Lymphoma Subtype Assay, was performed to identify the cell of origin in the DLBCL cases, in addition to Hans’s algorithm. Results: Using Hans’s algorithm, all DLBCL cases showed a germinal center-B-Cell subtype. The gene expression profile evaluated by the NanoString Lymphoma Subtype Assay revealed two cases of the GCB molecular subtype, while the others were unclassified. After first-line chemotherapy, 1 patient achieved complete remission, 3 were non-responders (2 died of lymphoma within 6 months, whereas the other achieved complete remission after autologous and allogeneic stem cell transplantation and is still alive). Conclusions: The origin of the second neoplastic cell in patients with DLBCL with a previous history of cHL remains controversial, although the different immunophenotypic characteristics suggest that it may mainly arise de novo in a subject with a possible individual predisposition to develop lymphoid neoplasms. Full article
(This article belongs to the Special Issue Haematological Neoplasms: Diagnosis and Management)
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