Incidence of hematologic cancer types in Japan by ICD-O-3 code: analysis of National Cancer Registry.

Aim: We classified subtypes of hematologic cancer in Japan's National Cancer Registry (NCR) in 2016 by ICD-O-3 code and compared numbers with the Japanese Society of Hematology's Blood Diseases Registry (BDR).Materials & methods: We reviewed data for individual cases, and calculated incidence per 100,000.Results: NCR reported 67,919 cases of hematological cancer, including diffuse large B-cell lymphoma, Not Otherwise Specified (NOS) (11.07 per 100,000), plasma cell myeloma (5.26/100,000), myelodysplastic syndrome, NOS (3.92/100,000), malignant lymphoma, NOS (3.03/100,000), marginal zone B-cell lymphoma, NOS (2.52/100,000) and follicular lymphoma, grade 1 (1.43/100,000). Around 48% of NCR cancers were also reported in the Blood Diseases Registry.Conclusion: ICD-O-3 codes revealed 12,365 additional cases to the 55,554 reported publicly. Accurately updated data are necessary for medical resource planning.

Japan's National Cancer Registry started in 2016, when 157 hematologic cancer subtypes and 67,919 cases (males: 37,916; females: 30,003) were reported, giving a cancer case incidence of 53.51 per 100,000. The most common hematologic cancers were diffuse large B-cell lymphoma, NOS (11.07 per 100,000), plasma cell myeloma (5.26 per 100,000) and myelodysplastic syndrome, NOS (3.92 per 100,000). Incidence data under ICD-O-3 by age and sex revealed different patterns among subtypes, allowing more accurate estimation of future patient numbers. This in turn allows grouping of indications to suit clinical needs, and aids pharmaceutical companies in understanding patient populations for new drugs.

Nanoscale insights into hematology: super-resolved imaging on blood cell structure, function, and pathology.

Fluorescence nanoscopy, also known as super-resolution microscopy, has transcended the conventional resolution barriers and enabled visualization of biological samples at nanometric resolutions. A series of super-resolution techniques have been developed and applied to investigate the molecular distribution, organization, and interactions in blood cells, as well as the underlying mechanisms of blood-cell-associated diseases. In this review, we provide an overview of various fluorescence nanoscopy technologies, outlining their current development stage and the challenges they are facing in terms of functionality and practicality. We specifically explore how these innovations have propelled forward the analysis of thrombocytes (platelets), erythrocytes (red blood cells) and leukocytes (white blood cells), shedding light on the nanoscale arrangement of subcellular components and molecular interactions. We spotlight novel biomarkers uncovered by fluorescence nanoscopy for disease diagnosis, such as thrombocytopathies, malignancies, and infectious diseases. Furthermore, we discuss the technological hurdles and chart out prospective avenues for future research directions. This review aims to underscore the significant contributions of fluorescence nanoscopy to the field of blood cell analysis and disease diagnosis, poised to revolutionize our approach to exploring, understanding, and managing disease at the molecular level.

[Removal of a dermoid cyst of the floor of the mouth in a patient with severe hemophilia A]. / Udalenie dermoidnoi kisty dna rta u bol'nogo tyazheloi formoi gemofilii A.

The article presents a case of a surgical treatment of removing a dermoid cyst of the floor of the oral cavity in a patient with severe hemophilia A. A detailed analysis was carried out of the surgical operation, postoperative management, coagulation factor replacement therapy and accompanying therapy, as well as the features of anesthesia, which allowed a surgical intervention without any hemorrhagic and infectious complications.

A review on acute phase response in parasitic blood diseases of ruminants.

Parasitic blood diseases (theileriosis, babesiosis, anaplasmosis, and trypanosomiasis) are common in regions where the distributions of the hosts, parasites, and vectors are convergent. They endanger animal production, and a few are also harmful to public health. The acute phase reaction (APR) is a complex, non-specific reaction that occurs in various events, including surgical trauma, infection, stress, inflammation, and neoplasia. To understand pathogenesis, we must study APR effects and acute phase proteins (APPs) alterations in naturally occurring and experimental infections. The elevation of haptoglobin (Hp), Serum amyloid A (SAA), and fibrinogen concentrations was markedly significant in bovine and ovine theileriosis. Hp, SAA, ceruloplasmin, and fibrinogen concentrations in anaplasmosis were dramatically elevated. A significant increase in SAA was observed in bovine babesiosis, while ovine babesiosis showed a significant rise in sialic acid levels. In cases of trypanosomiasis caused by T. vivax, there have been reports of elevated levels of Hp, complement C3, and antitrypsin. Improving our understanding of APR could result in more effective methods for diagnosis, treatment, control, and eradication of diseases. The article provides an overview of APPs alterations and other inflammation-related parameters (some cytokines, adenosine deaminase, and sialic acids) in parasitic blood diseases of ruminants.

Pleurisy as a Sign of Chronic Lymphocytic Leukemia.

The diagnosis of chronic lymphoid leukemia (CLL) is essentially based on a blood smear and immunophenotyping by flow cytometry of circulating lymphocytes. Unusual locations of the disease can sometimes be observed. Here we report the case of a patient admitted for the management of pleurisy. The pleural effusion was lymphocytic exudate; histological examination of the pleural biopsy along with immunohistochemistry helped yield the diagnosis of secondary localization of CLL. The patient was transferred to the Internal Medicine department where chemotherapy was introduced.

Diflovidazin damages the hematopoietic stem cells to zebrafish embryos via the TLR4/ NF-κB/ p53 pathway.

Exposure to environmental contaminants frequently induces the occurrence of blood diseases, but the underlying molecular mechanisms are scarcely known. The toxicity of Diflovidazin (DFD), a widely used mite-remover, to the blood system of non-target organisms requires urgent elucidation. To investigate the deleterious effects of DFD (2, 2.5, and 3 mg/L) on the development and survive of hematopoietic stem cells (HSCs), the zebrafish model was used in this study. DFD exposure reduced the number of HSCs and their subtypes, including macrophages, neutrophils, thymus T-cells, erythrocytes, and platelets. The significant changes in the abnormal apoptosis and differentiation of HSCs were the major reasons for the reduction in blood cells. Using small-molecule antagonists and p53 morpholino revealed that the NF-κB/p53 pathway was responsible for the apoptosis of HSCs upon DFD exposure. The restoration results attributed to the TLR4 inhibitor and molecular docking showed that the TLR4 protein, which was upstream of NF-κB signaling, played a vital role in DFD toxicology. This study elucidates the role and molecular mechanism of DFD in damaging zebrafish HSCs. It provides a theoretical basis for the occurrence of various blood diseases in zebrafish and other organisms.

The Electrophoretic Revolution in the 1960s: Historical Epistemology Meets the Global History of Science and Technology.

This paper uses zone electrophoresis, one of the most frequently used tools in molecular biology, to explore two ideas derived from Hans-Jörg Rheinberger's reflections on experiments. First, the constraining role played by technical objects-instrumentation and material conditions-in the production of knowledge or epistemic things. Second, the production of interconnected experimental systems by such technical objects, which results in the unexpected entanglement of research fields and experimental cultures. By the beginning of the 1960s, the inception of zone electrophoresis in laboratories around the world transformed-some say, revolutionized-the study of proteins. Even today, electrophoresis continues to open research venues and questions in biomedicine, molecular biology, human genetics, and in the field of molecular evolution. In my essay, I seek to look at the interconnected lives of zone electrophoresis and address the broader social, and even global context, in which this apparently humble technique became a salient tool in the production of biological knowledge. In so doing, I aim to take the past and present of the history and historiography of experimental systems to the future, where experiments and technologies are interrogated as they are used in different geographies and contexts, including contexts of poverty.

LGL leukemia patients exhibit substantial protective humoral responses following SARS-CoV-2 vaccination.

Large granular lymphocyte leukemia is a rare chronic lymphoproliferative disorder of cytotoxic cells. Other hematological malignancies such as CLL and multiple myeloma have been associated with poor vaccination response and markedly increased severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) mortality rates, specifically in patients who have undergone immunosuppressive therapy. Given the immunosuppressive therapies often used to treat the disease, large granular lymphocytic (LGL) patients may be especially vulnerable to SARS-CoV-2 infection. A questionnaire was sent to all patients in the LGL Leukemia Registry at the University of Virginia (UVA) to obtain information on vaccination status, type of vaccine received, side effects of vaccination, patient treatment status before, during, and after vaccination, antibody testing, history of coronavirus disease 2019 (COVID-19) infection, and presence or absence of booster vaccination. Antibody testing of 27 patients who had quantitative SARS-CoV-2 Spike Protein IgG levels determined by University of Virginia medical laboratories via the Abbott Architect SARS-CoV-2 IgG II assay were collected. The assay was scored as reactive at a threshold of ≥50.0 AU/mL or nonreactive with a threshold of <50.0 AU/mL. LGL patients without treatment as well as patients who held treatment prior to their vaccination have a robust humoral response to SARS-CoV-2 vaccines. Patients who did not hold their immunosuppressive treatments have signifigantly diminished vaccine response compared to those who held their immunosuppressive treatment. Our findings support a dual strategy of pausing immunotherapy during the vaccination window and administration of the SARS-CoV-2 booster to all LGL leukemia patients to maximize protective antibodies.

A Comprehensive Review on the Benefits and Problems of Curcumin with Respect to Human Health.

Curcumin is the most important active component in turmeric extracts. Curcumin, a natural monomer from plants has received a considerable attention as a dietary supplement, exhibiting evident activity in a wide range of human pathological conditions. In general, curcumin is beneficial to human health, demonstrating pharmacological activities of anti-inflammation and antioxidation, as well as antitumor and immune regulation activities. Curcumin also presents therapeutic potential in neurodegenerative, cardiovascular and cerebrovascular diseases. In this review article, we summarize the advancements made in recent years with respect to curcumin as a biologically active agent in malignant tumors, Alzheimer's disease (AD), hematological diseases and viral infectious diseases. We also focus on problems associated with curcumin from basic research to clinical translation, such as its low solubility, leading to poor bioavailability, as well as the controversy surrounding the association between curcumin purity and effect. Through a review and summary of the clinical research on curcumin and case reports of adverse effects, we found that the clinical transformation of curcumin is not successful, and excessive intake of curcumin may have adverse effects on the kidneys, heart, liver, blood and immune system, which leads us to warn that curcumin has a long way to go from basic research to application transformation.

The Impact of the COVID-19 Pandemic on the Organization of Cardio-Hematology Care-A Polish Single Center Experience.

Background and Objectives: We present a retrospective report on the cardio-hematological care of hematology patients at a university hospital in Poland during the COVID-19 pandemic. Materials and Methods: The number of hospitalizations at the Hematology Department and cardio-hematology consultations throughout 2019 and 2020 was analyzed. The types of cardiac procedures, risk factors, and complications were also assessed. Results: A significant reduction in the number of hospitalizations was observed in 2020 as compared to 2019. However, there were no significant differences in the incidence of hematological diseases between both of the analyzed years. In 2019, 299 cardiac consultations were performed in hematological patients, and there was a total of 352 such consultations performed in 2020 (p = 0.042). Less high-risk tests (transesophageal and stress echocardiography) were performed in 2020, in favor of the use of cardiac computed tomography in cardiac diagnostics as it was safer during the pandemic. At least one cardiovascular risk factor during cardiac consultation was noted in 42% and 48% of hematological patients in 2019 and 2020, respectively. Among 651 examined hematological patients, the most common findings were mild cardiac complications of hemato-oncological treatment, which were found in 57 patients. Conclusions: This study seems to confirm that during a pandemic there is an increased demand for well-organized cardio-hematology consultations.

Precursor B-cell acute lymphoblastic leukaemia-a global view.

As haematologists, we always seek to follow standardised guidelines for practice and apply the best treatment within our means for our patients with blood diseases. However, treatment can never follow an exact recipe. Opinions differ as to the best approach; sometimes more than one treatment approach results in identical outcomes, or treatments differ only by the manner in which they fail. Furthermore, the haematologist is faced with constraints relating to the local economic environment. Patients too are not the same the world over. Early presentation is commoner in the developed world, as is the patient's understanding of the disease process. This in turn has an impact on the way patients are managed, the rigorousness of patient adhesion to the treatment schedule and the outcome. Here we take a look at the precursor B-cell acute lymphoblastic leukaemia in an adolescent in a range of different settings from low- to high income countries with widely differing challenges for diagnosis, therpy and follow-up. For these reasons, given the same starting conditions, patients will be treated differently according to the institute and the country they are in. Experts from around the world have been tasked to describe their management plan and rationale for a specific disease presentation. Here they explore the management of precursor B-cell acute lymphoblastic leukaemia (pre-B ALL) in five different institutions worldwide with a focus on those with more or less strained economies. We end with a conclusion from an expert in the field comparing and contrasting these different management styles and considering their merits and limitations.

Base and Prime Editing Technologies for Blood Disorders.

Nuclease-based genome editing strategies hold great promise for the treatment of blood disorders. However, a major drawback of these approaches is the generation of potentially harmful double strand breaks (DSBs). Base editing is a CRISPR-Cas9-based genome editing technology that allows the introduction of point mutations in the DNA without generating DSBs. Two major classes of base editors have been developed: cytidine base editors or CBEs allowing C>T conversions and adenine base editors or ABEs allowing A>G conversions. The scope of base editing tools has been extensively broadened, allowing higher efficiency, specificity, accessibility to previously inaccessible genetic loci and multiplexing, while maintaining a low rate of Insertions and Deletions (InDels). Base editing is a promising therapeutic strategy for genetic diseases caused by point mutations, such as many blood disorders and might be more effective than approaches based on homology-directed repair, which is moderately efficient in hematopoietic stem cells, the target cell population of many gene therapy approaches. In this review, we describe the development and evolution of the base editing system and its potential to correct blood disorders. We also discuss challenges of base editing approaches-including the delivery of base editors and the off-target events-and the advantages and disadvantages of base editing compared to classical genome editing strategies. Finally, we summarize the recent technologies that have further expanded the potential to correct genetic mutations, such as the novel base editing system allowing base transversions and the more versatile prime editing strategy.

Low-affinity immunoglobulin gamma Fc region receptor III-B (FcγRIIIB, CD16B) deficiency in patients with blood and immune system disorders.

Low-affinity immunoglobulin gamma Fc region receptor III-B (FcγRIIIB) deficiency is present in ˜0·05% of the general population. Among our patients, FcγRIIIB deficiency was less frequent in those with immune-system disorders (one of 1815 patients, 0·05%) than in those with blood disorders (nine of 2147 patients, 0·42%, P = 0·023): mainly primary immune thrombocytopenia (4·34%), therapy related myeloid neoplasms (1·16%) and myelodysplastic syndrome with excess blasts (1·28%). Four of the nine (44·4%) patients with blood disorders were diagnosed with or quickly evolved to acute myeloid leukaemia (AML), suggesting that FcγRIIIB deficiency could be an adverse prognostic factor for progression to AML that should be confirmed in large multicentre studies.

Zebrafish blood disease models and applications.

Hematopoiesis is a complex, orderly and conserved developmental process, coordinated by multiple factors including transcription factors and signaling pathways. Dysregulation of any of these factors may cause developmental or functional defects in the blood system, leading to the pathogenesis of blood diseases. Zebrafish hematopoiesis and the underlying molecular mechanisms are highly conserved with those in mammals. The use of zebrafish to recapitulate abnormal changes in pathogenic factors can build models of related blood diseases, thus providing powerful tools for exploring the molecular mechanisms of pathogenesis and progression, visualization of tumorigenesis and high-throughput chemical screening. In this review, we summarize the zebrafish models of blood diseases and their applications. These disease models not only help to improve our understanding of the pathophysiology of the blood system and the molecular mechanisms on pathogeneses of blood diseases, but also provide new ideas for the treatment of clinically relevant hematological malignancies.

[Comparative analysis of RDW variability depending on belonging to a defined nosological group of diseases according to ICD-10 classification.] / Сравнительный анализ вариабельности индекса гетерогенности эритроцитов в зависимости от принадлежности к определенной нозологической группе болезней по классификации МКБ-10.

The aim of this study was to investigate RDW variability and conduct a comparative analysis of the RDW level in patients depending on their belonging to a defined nosological group of diseases according to the ICD-10 classification. All patients who complete blood count tests in our hospital from January to December 2016 were included in the study. The identification of the patient's belonging to a specific nosological group according to the ICD-10 classification was carried out on the basis of the disease analysis code indicated in the direction of the blood test. 8056 patient records were included in the final analysis. Deviations beyond the upper reference range for the RDW indicator (> 14.5%) in this study were identified for patients of the following nosological groups according to ICD-10: C - neoplasms; D - blood diseases; S - injuries; T - poisoning. Significant intergroup differences were obtained according to the Kruskal-Wallis rank analysis of variance (Kruskal-Wallis test: H (19, n= 7622) = 214.9672 p = 0.0000). According to the results of this study, we can conclude that RDW has specificity for certain diseases (neoplasms; blood diseases; injuries; poisoning). In case of cardiovascular and other diseases, deviations of this indicator beyond the limits of the upper reference values were not found in this study.

Incidence of haematological malignancies in adults in Szabolcs-Szatmár-Bereg county, Hungary. Analysis of data of a 36-year period / A felnottkori malignus hematológiai megbetegedések gyakorisága Szabolcs-Szatmár-Bereg megyében. 36 év adatainak elemzése.

INTRODUCTION: During a 36-year period (between January 1, 1983 and December 31, 2018), 5159 adult patients with newly diagnosed haematological malignancy were registered in the leukaemia/lymphoma registry of Szabolcs-Szatmár-Bereg county. AIM: The review of the incidence of different haematological malignancy in the authors' county, and the changes of incidence from time to time, the associated haematological malignancies, and familial occurrence of malignant haematological diseases. METHOD: Detailed analysis of the data of the registry, with statistical analysis of incidence. RESULTS: The incidence of Hodgkin disease and non-Hodgkin's lymphoma (1.49 and 7.12 new cases, respectively/100 000 inhabitants/year) was a little smaller, that of essential thrombocythaemia was larger than in the published data. The incidence of all other haematological malignancies corresponded to the data of the literature. The change of incidence of all malignant haematological diseases was similar to the published data. In the registry, there were 35 patients with two different malignant haematological diseases appearing simultaneously or successively. During the 36-year period, 88 families with haematological malignancies were recorded in the registry. CONCLUSION: With the exception of Hodgkin disease, non-Hodgkin's lymphoma, and essential thrombocythaemia, the incidence of other haematological malignancies corresponded to the data of the literature. The change of incidence in all entities was similar to that observed by other authors. The authors in their country do not know other published data related to associated malignant haematological diseases. The observed anteposition in familial haematological diseases of uncle/aunt and nephew/cousin, and anteposition in malignant haematological diseases of siblings are equally new in the literature. Orv Hetil. 2020; 161(34): 1400-1413.

Nanoparticles for hematologic diseases detection and treatment.

Nanotechnology, as an interdisciplinary science, combines engineering, physics, material sciences, and chemistry with the biomedicine knowhow, trying the management of a wide range of diseases. Nanoparticle-based devices holding tumor imaging, targeting and therapy capabilities are formerly under study. Since conventional hematological therapies are sometimes defined by reduced selectivity, low therapeutic efficacy and many side effects, in this review we discuss the potential advantages of the NPs' use in alternative/combined strategies. In the introduction the basic notion of nanomedicine and nanoparticles' classification are described, while in the main text nanodiagnostics, nanotherapeutics and theranostics solutions coming out from the use of a wide-ranging NPs availability are listed and discussed.

Epidemiology of malignant hemopathies recorded in hospitals in Cameroon.

Data about malignant blood diseases are sparse in Cameroon. Their epidemiology was studied in patients at the General Hospital of Douala (GHD) and the Yaoundé Central Hospital (CHY) from 2004 through 2014. The variables we studied were social and demographic (age, sex, occupation, marital status), clinical (reasons for consultation, clinical signs, year of diagnosis), and biological (blood count, myelogram and blood smear, immunophenotyping, biopsy, and cytogenetics). In all, 4409 files were reviewed and 454 cases identified, documented and confirmed (248 in GHD and 206 in CHY). The prevalence of malignant blood diseases was 10.4%. The patients' mean age was 44.3 ± 19 [range : 1-80] years and the M/F sex ratio 1.4/1. In 32.2% of the cases, the patient consulted because of a tumor. The most frequent malignant blood diseases, in decreasing order, were non-Hodgkin's lymphoma (31.1%), chronic myeloid leukemia (21.4%), chronic lymphoid leukemia (12.6%), multiple myeloma (11.2%), acute lymphoblastic leukemia (7.4%), and acute myeloblastic leukemia (6.4%). Their incidence by age group showed that acute lymphoblastic leukemia was most common among children (20%), and chronic myeloid leukemia among young adults (28.9%). The main hemogram abnormalities were anemia (73.7%), hyperleukocytosis (57.3%), and thrombopenia (39.2%). Various types of malignant blood diseases thus exist in the hospital environment in Cameroon, and their forms are underdiagnosed.

[Anteposition in malignant hematologic diseases of siblings in Szabolcs-Szatmár-Bereg county, Hungary. Analysis of data of a 34-year period]. / Antepozíció testvérekben észlelt malignus hematológiai betegségekben. A 34 éves Szabolcs-Szatmár-Bereg megyei leukaemia/lymphoma regiszter adataiból levonható következtetések.

INTRODUCTION: In their previous works, the authors reported findings from familial hematologic malignancies in Szabolcs-Szatmár-Bereg county, Hungary. So far there are no other studies on this topic available in Hungary. AIM: Detailed analysis of epidemiologic features of hematologic malignancies of siblings. METHOD: During a 34-year period (between January 1, 1983 and December 31, 2016), 86 families with hematologic malignancies were recorded in Szabolcs-Szatmár-Bereg county. Among them, 19 cases of the affected siblings were registered. RESULTS: In one family there were three sisters with polycythaemia vera, hence the number of analysed disease associations was 21. In all of the 21 cases, the younger sibling's disease developed earlier. The average anteposition was 10.8 (1-33) years (median: 10 years). CONCLUSION: The anticipation was earlier observed in multigeneration hematological malignancies between direct and collateral descendants. On the basis of the above data, anteposition of the disease was observed in younger siblings. Orv Hetil. 2017; 158(33): 1283-1287.