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Twenty-five years of epidemiological recording on myeloid malignancies: data from the specialized registry of hematologic malignancies of Cote d'Or (Burgundy, France).

BACKGROUND: Epidemiological data on myeloid malignancies are very rare in the literature due to a lack of registration by cancer registries until 2000. The Registry of Hematologic Malignancies of the Côte d'Or Department in France has, however, steadfastly registered data on cases occurring in the Department since 1980, resulting, to date, in a database of over 5,000 cases classified according to the ICD-O-3 classification, following the most recent World Health Organization classification criteria. DESIGN AND METHODS: Twenty-five years of data on myeloid malignancies, including acute myeloid

[Population-based incidence of lymphoid neoplasms by histological subtypes in Girona (Spain), 1994-2001].

BACKGROUND AND OBJECTIVE: We aimed to assess the distribution of the lymphoid neoplasms and their histological subtypes in accordance with the World Health Organization (WHO) classification by calculating their incidences rates in our area. PATIENTS AND METHOD: From January 1994 to December 2001, 1,288 patients diagnosed with lymphoid neoplasms were recruited in the population-based Cancer Registry of Girona. Former pathological and hematological diagnoses were reviewed and some were prospectively reclassified following the latest WHO classification. RESULTS: Following criteria established b

Plasmacytoid dendritic cell proliferations, malignant histiocytic (macrophage/dendritic cell) neoplasms and follicular dendritic cell neoplasms: report from the 2024 Joint CSHP/EA4HP/SH workshop.

Histiocytic and dendritic cell neoplasms of hematopoietic origin are derived from or show differentiation towards the cells of the mononuclear phagocyte system. Plasmacytoid dendritic cell proliferations/neoplasms include mature plasmacytoid dendritic cell proliferations identified in the context of a defined myeloid neoplasm and blastic plasmacytoid dendritic cell neoplasm, an aggressive tumor with a poor outcome. Malignant histiocytic (macrophage/dendritic cell) neoplasms occur de novo or in the setting of another hematological malignancy, typically "transdifferentiated" from associated lymp

Concomitant Clonal CBFB::MYH11 and PDGFRB Fusions in a Case of De Novo Acute Myeloid Leukemia.

Background: Acute myeloid leukemia (AML) with CBFB::MYH11 fusion and myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene f … This case illustrates the diagnostic challenges posed by concomitant class-defining alterations in hematologic neoplasms and underscores the importance of integrated genomic assessment.

Updates on Langerhans cell histiocytosis and other histiocytosis in children: invited review-challenges and novelties in paediatric tumours.

Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG) family lesions, and Rosai-Dorfman-Destombes disease (RDD) are now classified by the World Health Organization (WHO) under the heading of histiocytic/dendritic cell neoplasms. Each disease may manifest as a focal lesion, as multiple lesions, or as a widespread aggressive systemic disease with visceral organ involvement. Erdheim-Chester disease (ECD) is a rare systemic disease process of adults with limited cases in children. Challenges in diagnosis and novel disease presentations, including ALK-positive histiocytosis (a newly r

Guide to the Diagnosis of Myeloid Neoplasms: A Bone Marrow Pathology Group Approach.

OBJECTIVES: The practicing pathologist is challenged by the ever-increasing diagnostic complexity of myeloid neoplasms. This guide is intended to provide a general roadmap from initial case detection, often triggered by complete blood count results with subsequent blood smear review, to final diagnosis. METHODS: The integration of hematologic, morphologic, immunophenotypic, and genetic features into routine practice is standard of care. The requirement for molecular genetic testing has increased along with the complexity of test types, the utility of different testing modalities in identifyin

Increase in circulating CD4⁺CD25⁺Foxp3⁺ T cells in patients with Philadelphia-negative chronic myeloproliferative neoplasms during treatment with IFN-α.

Recent reports have described complete or major molecular remission in patients with polycythemia vera after long-term treatment with the immunomodulatory agent IFN-α2. Accordingly, there are reasons to believe that the immune system is a key player in eradicating the JAK2 mutated clone in these patients. Foxp3(+) regulatory T cells play a pivotal role in maintaining immune homeostasis and, importantly, preventing immune reactivity to self-antigens; however, their suppressive activity can compromise an effective antitumor immune response, and high frequencies of regulatory T cells in periphera

The clinical spectrum of lymphangiomas and lymphangiomatosis of the spleen.

Lymphangiomas of the spleen are infrequent neoplasms of the spleen with clinical manifestations ranging from insignificant incidental findings to large, symptomatic cystic masses requiring surgical intervention. An associated syndrome may be lymphangiomatosis, in which the lymphangiomatous process involves other sites or organs, such as the liver, spleen, axilla, and mediastinum. Vasoformative neoplasms (hemangioma, lymphangioma) are relatively uncommon tumors or hamartomas of the spleen. Although well reported in the literature, their surgical significance is not well recognized. These lesion

Refining the relationship between gut microbiota and common hematologic malignancies: insights from a bidirectional Mendelian randomization study.

BackgroundThe relationship between gut microbiota and hematologic malignancies has attracted considerable attention. As research progresses, it has become increasingly clear that the composition of gut microbiota may influence the onset and progression of hematologic malignancies. However, our understanding of this association remains limited.MethodsIn our study, we classified gut microbiota into five groups based on information at the phylum, class, order, family, and genus levels. Subsequently, we obtained data related to common hematologic malignancies from the IEU Open GWAS project. We the

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… This suggested that the associations between other hematologic neoplasms and gut microbiota may have been more consistent and less influenced by unknown factors. …

LYmphoid NeXt-Generation Sequencing (LYNX) Panel: A Comprehensive Capture-Based Sequencing Tool for the Analysis of Prognostic and Predictive Markers in Lymphoid Malignancies.

B-cell neoplasms represent a clinically heterogeneous group of hematologic malignancies with considerably diverse genomic architecture recently endorsed by next-generation sequencing (NGS) studies. Because multiple genetic defects have a potential or confirmed clinical impact, a tendency toward more comprehensive testing of diagnostic, prognostic, and predictive markers is desired. This study introduces the design, validation, and implementation of an integrative, custom-designed, capture-based NGS panel titled LYmphoid NeXt-generation sequencing (LYNX) for the analysis of standard and novel m

Industries and cancer.

Epidemiologic evidence on the relationship between selected industries and cancer is reviewed. This article will focus on several industries … to polychlorinated biphenyls (PCBs) have reported excess mortality from gastrointestinal neoplasms, hematologic neoplasms, and skin cancer (specifically malignant melanoma); IARC considers that the evidence for carcinogenicity in humans is 'limited.' Employment in the boot and shoe industry has been associated with nasal adenocarcinomas in England and Italy ('sufficien …

Optimizing Polycythemia Vera Management Through a Collaborative Care Model Between Leukemia Oncologists and an Integrated Specialty Pharmacy.

Polycythemia vera (PV) is a chronic myeloproliferative neoplasm that requires long-term hematologic control and symptom monitoring. Despite advanced therapies and treatment guidelines, real-world adherence to therapeutic goals and routine symptom assessment remains inconsistent, particularly outside the academic setting. To optimize care, a pharmacist-led clinical support program was implemented at Levine Cancer Institute, a large regional hematologic malignancy center, in collaboration with the integrated institutional Atrium Health Specialty Pharmacy Service (SPS). This program aimed to stan

Inhibition of NEDDylation enhances the cytostatic effect of Rohinitib on chronic lymphocytic leukemia cells.

Chronic lymphocytic leukemia (CLL) is an incurable hematologic neoplasm that primarily affects elderly individuals. Its treatment consumes a … e specific to CLL, as it is not significantly relevant in healthy lymphocytes or in other prevalent hematologic neoplasms, such as multiple myeloma. A molecular characterization of this synergistic effect was conducted, revealing RNA translation dysregulation of NAE1 and UBE2M, as well as kinases involved in BCR signaling, in CLL tumor cells. Additionally, a cooperativ …

[Hospital readmission rate and associated factors among health services enrollees in Colombia].

Hospital readmissions are common and expensive, and there is little information on the problem in Colombia. The objective was to determine the frequency of 30-day all-cause hospital readmissions and associated factors. This was a retrospective analytical cohort study of 64,969 hospitalizations from January 2008 to January 2009 in 47 Colombian cities. 6,573 hospital readmissions, prevalence: 10.1% (men 10.9%, women 9.5%), 44.7% > 65 years of age. Hospital readmissions was associated with higher mortality (5.8% vs. 1.8%). There was an increase in the Hospital readmissions rate in patients with d

Tumor-forming plasmacytoid dendritic cells associated with myeloid neoplasms. Report of a peculiar case with histopathologic features masquerading as lupus erythematosus.

Plasmacytoid dendritic cells (PDC) belong to a subtype of dendritic cells that are normally absent in healthy skin. In some inflammatory diseases of the skin, especially lupus erythematosus (LE), these cells are occasionally recruited in great amounts, which can be used as a helpful clue for diagnosis. Rarely, PDC may also accumulate in the skin of patients with myeloid leukemia, a yet poorly known condition currently called 'tumor-forming PDC associated with myeloid neoplasms'. In this study, we describe a patient with unsuspected chronic myelomonocytic leukemia who developed cutaneous lesion

Canakinumab or emavusertib for anemia in lower-risk MDS: results of the CANFIRE and LUCAS phase 2, open-label, multicenter trials.

BACKGROUND: Dysregulated innate immune signaling promotes inflammatory suppression of hematopoietic stem and progenitor cell function, contributing to ineffective erythropoiesis in myelodysplastic neoplasms (MDS). The phase 2, open-label CANFIRE and LUCAS trials aimed to determine the efficacy and safety of canakinumab or emavusertib, respectively, in patients with lower-risk MDS (LR-MDS) or myelodysplastic/myeloproliferative neoplasms (MDS/MPN). METHODS: Patients in CANFIRE were refractory, intolerant to, or ineligible for erythropoiesis-stimulating agent (ESA) treatment and received canakin

Evaluation of clinical and laboratory findings with JAK2 V617F mutation as an independent variable in essential thrombocytosis.

Essential thrombocythemia (ET) is an entity of classic Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), characterized by thrombocytosis with megakaryocytic hyperplasia and thrombocytes are increased with abnormal functions. Discovery of the protein tyrosine kinase JAK2 V617F allele contributed to better understanding of the pathogenetic mechanisms of MPNs. Acquired single point mutation in the JAK2 V617F was determined approximately 50-60 % of patients with ET. In this study we aimed to investigate the relationship between JAK2 V617F gene mutation, hematologic, biochemical

Incidence, Prevalence, and Survival Outcomes of Patients with Myeloproliferative Neoplasms in the United States: a SEER database analysis, years 2000-2021.

Myeloproliferative Neoplasms (MPNs) are rare hematologic neoplasms. The epidemiology and outcomes of these rare neoplasms warrant an update considering new diagnostic tools and advances in treatment options. NCI's Surveillance, Epidemiology and End Result (SEER)-17 registries were interrogated to analyze adult patients diagnosed with MPN [ICD-O-3 codes: polycythemia vera (PV) 9950/3, essential thrombocythemia (ET) 9962/3, primary myelofibrosis (PMF) 9961/3, and chronic myeloid leukemia (CML) 9875/3] during the years 2000 to 2021. Data of 63,242 (ET, n=24 172; PV, n= 23 456; PMF, n=6 131; CML, …

Flow Cytometric Bone Marrow Evaluation in Suspected Myelodysplastic Neoplasms.

Myelodysplastic neoplasms (MDS) are acquired heterogeneous clonal hematopoietic stem cell neoplasms, clinically characterized by progressively ineffective hematopoiesis and an increased risk of acute myeloid leukemia. MDS are accompanied by an inflammatory microenvironment and genome instability. Signs of dysplasia can occur in the erythroid, myeloid, monocytic, and megakaryocytic cell lineages and result in anemia, neutropenia, and thrombocytopenia. Multi-parameter flow cytometry can be used to detect aberrant antigen expression patterns typical of MDS, which correlate with cytomorphologicall

Frequency of JAK2V617F, MPL and CALR driver mutations and associated clinical characteristics in a Norwegian patient cohort with myeloproliferative neoplasms.

Myeloproliferative neoplasms are hematological disorders characterized by increased production in one or more myeloid cell lines, associated with driver mutations in JAK2-, MPL- and CALR-genes. The aims of this study were to investigate the prevalence of these driver mutations in a Norwegian patient cohort with myeloproliferative neoplasms, and to assess whether the different mutations were associated with different clinical presentation and natural history.Results from 820 patients in whom analysis for JAK2V617F-, CALR- and MPL had been performed at Haukeland University Hospital in the period