Research Progress on Leucine-Rich Alpha-2 Glycoprotein 1: A Review

Leucine-rich alpha⁃2 glycoprotein 1 (LRG1) is an important member of the leucine-rich repetitive sequence protein family. LRG1 was mainly involved in normal physiological activities of the nervous system, such as synapse formation, synapse growth, the development of nerve processes, neurotransmitter transfer and release, and cell adhesion molecules or ligand-binding proteins. Also, LRG1 affected the development of respiratory diseases, hematological diseases, endocrine diseases, tumor diseases, eye diseases, cardiovascular diseases, rheumatic immune diseases, infectious diseases, etc. LRG1 was a newly discovered important upstream signaling molecule of transforming growth factor⁃β (TGF⁃β) that affected various pathological processes through the TGF⁃β signaling pathway. However, research on LRG1 and its involvement in the occurrence and development of diseases was still in its infancy and the current studies were mainly focused on proteomic detection and basic animal experimental reports. We could reasonably predict that LRG1 might act as a new direction and strategy for the treatment of many diseases.

ASSESSMENT OF CONGENITAL NEUTROPENIA IN CHILDREN: COMMON CLINICAL SCENERIES AND CLUES FOR MANAGEMENT

A disparate group of rare hematological diseases characterized by impaired maturation of neutrophil granulocytes defines congenital neutropenias. Neutropenic patients are prone to recurrent infections beginning in the first months of life. Of interest is “cyclic neutropenia”, an ultra-rare disorder revealed by sinusoidal variations of the neutrophil count and periodically-recurring infections every 21 days. Diagnosis of these disorders is frequently obscured by the multiple causes of recurrent fevers in children. Aim of this overview is to outline the physical assessment of children presenting with early-onset symptomatic neutropenia, identify the disease between the many medical conditions and even emergencies which should enter in differential diagnosis, hint at the potential management with granulocyte-colony stimulating factor, define the risk of evolution to hematologic malignancy, and summarize inter-professional team strategies for improving care coordination and outcomes of such patients.

Main aspects of etiopathogenesis, diagnosis and treatment of subhyaloid hemorrhages

Purpose. To present modern literature data on the main aspects of etiopathogenesis, diagnosis and treatment of subhyaloid hemorrhages. Material and methods. To perform the review, the search for literature sources on PubMed abstract databases was analyzed by the keywords «subhyaloid hemorrhages», «posterior hyaloidotomy» according to Russian publications on the topic of the article for the period up to 2020 inclusive. Results. Subhyaloid hemorrhage is a suspension of blood in a space bounded by a mesh membrane and a detached posterior hyaloid membrane. The most common cause of subhyaloid hemorrhages are retinal vascular diseases: diabetic retinopathy of the retina (30%), Valsalva hemorrhagic retinopathy (30%), central retinal vein thrombosis (20%). Subhyaloid hemorrhages are less common in hematological diseases, eye injury, rupture of the macroaneurysm, Terson syndrome. It has been established that the main complications due to untimely or inadequate treatment of subhyaloid hemorrhages are the formation of a premacular fibrous membrane, vitreoretinal fusion, traction retinal detachment, hemophthalmos, fibrosis and retinal atrophy, which require the right tactics in treatment. Conclusion. Thus, due to the prolonged presence of blood and fibrin in the premacular subhyaloidal space, there is a danger of mechanical and toxic effects on the retinal neuroepithelium of the macular region, which can lead to an irreversible decrease in visual functions. Key words: subhyaloid hemorrhages, Nd:YAG laser, posterior hyaloidotomy

The prevalence of bloodborne infections (hepatitis B and C, human immunodeficiency virus) among pediatric patients with oncological and hematological diseases and immunodeficiencies

Patients with oncological and hematological diseases are at high risk of acquiring bloodborne infections due to multiple blood transfusions and frequent parenteral exposure. In order to evaluate the prevalence of bloodborne infections (i. e., hepatitis B, hepatitis C, and human immunodeficiency virus (HIV)), we analyzed data on the seroprevalence of these viruses in patients admitted to the D. Rogachev NMRCPHOI from 2014 to 2020. We also performed a comparative analysis between these data and the prevalence of these infections in the total child population in Russia. The study was approved by the Independent Ethics Committee and the Scientific Council of the D. Rogachev NMRCPHOI. Among patients admitted to the D. Rogachev NMRCPHOI, the mean seroprevalence was 1.7% for hepatitis C, 0.2% for hepatitis B, and 0.1% for HIV. The seroprevalence of hepatitis B and C among our patients was 6 and 50 times higher than the prevalence among Russian children and adolescents, respectively. The prevalence of HIV among patients treated at the D. Rogachev NMRCPHOI was 3 times higher than that among the child and adolescent population in Russia. In patients with oncological and immunological diseases, the detection of HBV DNA, HCV RNA, and Anti-HBc is considered clinically useful and plays an important role in the diagnosis of occult hepatitis infections which cannot be identified with routine diagnostic tests. Our study with pediatric patients with oncological, hematological, and immunological diseases highlights the problem of nosocomial transmission of bloodborne pathogens. HCV transmission in medical facilities is the most pressing issue that requires the implementation of healthcare programs aimed at preventing parenteral transmission and at ensuring the safety of donated blood.

Trichosporon inkin fungemia case report: clinical and laboratory management

Trichosporon species are emerging as opportunistic pathogens that mainly affect immunocompromised patients. Patients with onco-hematological diseases usually present with fungemia by Trichosporon species, especially by T. asahii. Reports of this infection by other species of the genus are uncommon. Thus, in this paper, we present a case of T. inkin fungemia in a 39-year-old female patient with intestinal obstruction and absence of malignant hematological diseases. The late mycological diagnosis, the ineffective control of her pre-existing conditions and consequent failure to start antifungal therapy were the contributing factors for the patient's death.

Clonal hematopoiesis and its role in the development of hematological diseases

Introduction. The formation of blood cells in a healthy individual is ensured by polyclonal hematopoiesis. Recent studies have shown that with age, large clones with a common genetic marker are found in the peripheral blood, i. e. cells originating from a single progenitor cell. This phenomenon is called clonal hematopoiesis. In some cases, people with clonal hematopoiesis develop hematological diseases.Aim — to describe and summarize current data on the relationship between clonal hematopoiesis and hematological diseases.Main findings. This review describes the history of detection of clonal hematopoiesis, its main properties, the most frequent mutations in hematopoietic clones associated with the risk of transformation into myelodysplastic syndrome, and acute myeloid leukemia. The meaning and possible pathogenesis of tumor transformation are discussed.

A Large Cohort Study of SARS-CoV-2 Detection in Saliva: A Non-Invasive Alternative Diagnostic Test for Patients with Bleeding Disorders

Diagnosis of SARS-CoV-2 infections is mostly based on the nasopharyngeal swabs (NPS). However, this collection is invasive and uncomfortable, especially for children and patients with coagulopathies, whose NPS collection often causes bleeding. Thus, the aim of this study was to evaluate the usefulness and accuracy of saliva for the diagnosis of COVID-19 in patients presenting bleeding disorders. Samples of NPS, oropharyngeal swabs (OPS), and saliva were collected simultaneously from 1159 hospitalized patients with hematological diseases and from 524 healthcare workers, both symptomatic and asymptomatic for SARS-CoV-2. All samples were evaluated for SARS-CoV-2 by qRT-PCR. SARS-CoV-2 was detected in NPS, OPS and saliva from 16.9%, 14.4% and 15.6% individuals, respectively. Tests in saliva showed sensitivity, specificity, and overall agreement of 73.3%, 96.9% and 92.7% (=0.74), respectively. Salivary tests had good accuracy (AUC = 0.7) for discriminating negative and positive qRT-PCR for SARS-CoV-2. Higher sensitivity was observed in symptomatic than in non-symptomatic patients, as well as in healthy subjects than in patients with hematological disease, in both OPS and saliva. The mean viral load in NPS was significantly higher than in OPS and in saliva samples (p < 0.001). Saliva is a good diagnostic tool to detect SARS-CoV-2, especially among patients symptomatic for COVID-19, and is a valuable specimen for mass screening of hospitalized patients with hematological diseases, especially for those that with bleeding disorders.