Hematological Pathologies Research Articles

SPLENULE FREQUENCY ON COMPUTED TOMOGRAPHY SCANS IN CHILDREN, PRESENTING TO THE EMERGENCY DEPARTMENT

Objectives: Accessory spleen, also known as “splenule”, is the presence of splenic tissue in ectopic localisations. The presence of splenule is important, especially in patients planned for splenectomy, as it may cause refractory symptoms. The aim of the present study is to define the frequency of splenule(s) in children (0-17 years) who received non-contrast and contrast enhanced computed tomography (NECT and CECT) protocols in the emergency department.
 Material and methods: 748 children (aged 0 to 17 years) who were admitted to the emergency department between May 2015 – September 2022 and had NECT and CECT abdominal scans were included in the study. Patients whose CT protocols were incomplete and cases with traumatic splenic injury and / or cases with poor image quality and patients with a history of splenectomy or hematologic pathology were excluded from the study (n: 100). A total of 648 patients were included in the cohort. NECT and CECT scans of all patients were assessed; the localisation of splenules as well as the antero-posterior (AP), medio-lateral (ML) and cranio-caudal (CC) dimensions of each splenule were measured.
 Results: A total of 648 cases with 467 males (72.1%) and 181 females (27.9%) were included in the study. Splenules were observed in 131 (20.2%) cases. More than one splenule was observed in 21 of these 131 cases. A total number of 159 splenules were observed in total, with a mean volume of 0,72 ±0,95 ml. The most common location was found to be the splenic hilus (n=55, 41.9%). 
 Conclusion: Our study have stated that splenules are common anatomical variants, seen at a rate of 20,2% in this age cohort. A cross-sectional imaging should be performed to determine the presence, location, and number of the splenules before a planned splenectomy.

Clonal haematopoiesis of indeterminate potential in patients with venous thromboembolism.

Over the last decade, the concept of Clonal haematopoiesis of undetermined potential (CHIP) has emerged. Low frequency somatic mutations in hematopoietic cells can occur with age and might allow formation of clones in individuals with no characterized haematological pathology. These CHIP mutations are associated with an increased risk of cancer or atherothrombosis, and their prevalence are more and more studied in pathologies with an inflammatory component. In our study, we analysed, by next generation sequencing, the prevalence of CHIP mutation in 94 patients with deep venous thrombosis (DVT), distinguishing two clinical phenotypes: provoked distal and non-provoked proximal DVTs. We show that there is no difference in CHIP prevalence between these two groups, nor with a matched-aged control group. The number of mutation per patients and the affected genes remain also the same between the three groups. Consequently and despite the relative small number of patients in each cohort, it seems that CHIP is not a strong concern in venous thromboembolism.

Exploring links between toxic-environmental factors and hematologic malignancies: considerations for data-driven health decision making

Introduction: some toxic-environmental factors are frequently related to malignant hematological diseases, which have increased in recent years.Objective: to describe the frequency of appearance of toxic-environmental factors in patients diagnosed with hematological neoplasms.Method: a study was conducted cross-sectional descriptive study in patients diagnosed with hematological pathologies in the year 2020, a structured interview was applied to them looking for toxic-environmental factors, occupation, contact with toxic substances, origin, drinking water, toxic habits, diet, among others. Patients who used pesticides were given a second interview that collected: the type of substance, exposure time, time since last exposure, history of poisoning, protection measures, knowledge and application of the same. Results: patients with lymphoma predominated. non-Hodgkin (25,5 %), ages between 61-80 years (50 %), farmers (31 %), rural origin (57 %), those who had contact with toxins (64,4 %). The most used toxin was pesticides, the average exposure time was 14 years, the last contact 9,2 years, protection measures are used only sometimes, their need is unknown and 90 % do not know any protection measure. Conclusions: according to the results it is necessary to create strategies to guide the population about this risk and how to modify it

P479 PATIENT WITH REGRESSION MULTIPLE MYELOMA AND INFILTRATIVE HYPERTROPHIC CARDIOMYOPATHY OF NDD UNDERGO CARDIAC TRANSPLANT

Abstract Introduction We report the case of a 52–year–old patient with a history of multiple myeloma undergoing chemotherapy treatment according to the DaraRD scheme and currently in remission. Associated with haematological pathology, infiltrative hypertrophic cardiomyopathy of ndd is highlighted. Following haematological and instrumental screening tests, the patient underwent a heart transplant for frequent exacerbations of heart failure. Case Report A 52–year–old patient, suffering from multiple myeloma for about 1 year, for which he began chemotherapy treatment with DaraRD scheme and good response to therapy. The patient also presented dyspnoic symptoms and declining edema with echocardiographic finding of infiltrative hypertrophic heart disease for which he undergoes diagnostic investigations. He performs: • Serial echocardiograms which showed marked uniform hypertrophy with ground glass appearance of the left ventricle and severely reduced systolic function; • PET–CT with no hyperaccumulation of the radiopharmaceutical; • Cardiac MRI with evidence of severely hypocontractile left ventricle and wall changes which could be associated with amyloid infiltration; • myocardial scintigraphy that does not give evidence for the deposit of the amyloid substance of the ATTR type in the heart; • biopsy of umbilical fat and cardiac biopsy, both negative for deposition of substance AL with Congo Red staining; • Right cardiac catheterization with evidence of post–capillary pulmonary hypertension. The patient was subjected to several exacerbations of heart failure which required continuous infusion therapy with inotropes and, ultimately, heart transplantation. At the time of surgery, the heart appeared enlarged, extremely hypocontractile and with rigid walls, with poor compliance of the ventricular muscle wall. The patient responded well to the surgical procedure, in the absence of signs and symptoms of rejection, with improvement of general clinical condition. Conclusions At the end of the surgical procedure and the rehabilitation period, the patient was discharged home, without showing signs and symptoms of graft rejection or multiple myeloma exacerbations, in stable and satisfactory general clinical conditions.

P423 RELAPSE OF ACUTE MYELOID LEUKEMIA WITH CARDIAC INFILTRATION: A CASE REPORT

Abstract We describe the case of a 42–year–old man who presented to the emergency department suffering from asthenia, dyspnea and tachycardia. The patient had been diagnosed, two years earlier, with acute myeloid leukemia (AML), treated with two lines of chemotherapy and allogeneic bone marrow transplantation from a family donor. Atrial fibrillation with rapid ventricular response was documented at the ECG. The echocardiogram showed moderate left ventricular dysfunction in the presence of dishomogeneous thickening of left and right ventricular wall and a severe circumferential pericardial effusion causing cardiac tamponade, with the need of urgent pericardiocentesis. AML relapse was documented at the laboratory tests. The suspected cardiac involvement was investigated with cardiac MRI which documented thickening and diffuse late gadolinium enhancement at the level of the left and right ventricular wall, the interatrial septum and the atria walls. The pericardium and the epicardial fat were also thickened and endowed with contrast enhancement. A lymphocyte assay was performed on pericardial fluid and showed the presence of a blast population (approximately 27% of white blood cells) with variable expression of myeloid antigens. The clinical, radiological and finally immunophenotypic picture therefore concluded with relapse of acute myeloid leukemia with myocardial and pericardial infiltration. Acute myeloid leukemia usually presents with symptoms related to bone marrow failure. Clinically significant cardiac involvement is rare but should be considered when cardiorespiratory symptoms occur. Cardiac manifestations include coronary artery disease, heart failure, conduction disturbances, myocarditis, pericarditis, and pericardial effusion. Understanding the underlying pathophysiology and recognizing potential cardiac manifestations could help in the early diagnosis, evaluation, and treatment of these patients. Echocardiography or cardiac MRI could reveal morphological, structural and signal abnormalities of the cardiac structures and therefore should raise the suspicion of a possible myocardial infiltration by leukemia cells, both in patients with a positive history of leukemia and in those in whom cardiac involvement could represent the first manifestation of a hematological pathology.

Systematic review and meta-analysis of the clinical effectiveness of tixagevimab/cilgavimab for prophylaxis of COVID-19 in immunocompromised patients.

Immunocompromised patients, such as those with a haematological malignancy, are at higher risk of SARS-CoV-2 infection, severe outcomes and mortality. Tixagevimab/cilgavimab is a monoclonal antibody combination which binds to the SARS-CoV-2 spike protein. The PROVENT phase III clinical trial reported that tixagevimab/cilgavimab prophylaxis significantly reduced the risk of COVID-19 infection in immunocompromised participants. However, the trial was conducted before the Omicron variant became prevalent. This systematic review and meta-analysis provide an up-to-date summary of the real-world effectiveness of tixagevimab/cilgavimab in immunocompromised patients, including patients with haematological malignancies. Clinical studies from 1 January 2021 to 1 October 2022, which reported breakthrough COVID-19 infections after tixagevimab/cilgavimab, were included. COVID-19-related hospitalisations, intensive care admissions and mortality were also assessed. A meta-analysis was performed to ascertain overall clinical effectiveness. Eighteen studies, with 25 345 immunocompromised participants, including 5438 patients with haematological pathologies, were included in the review. The overall clinical effectiveness of tixagevimab/cilgavimab against COVID-19 breakthrough infection, hospitalisation, intensive care admission and COVID-19-specific mortality was 40.54%, 66.19%, 82.13% and 92.39%, respectively. This review highlights the clinical effectiveness of tixagevimab/cilgavimab at reducing COVID-19 infection and severe outcomes for immunosuppressed individuals, including patients with a haematological malignancy, during the Omicron-predominant era. Real-world studies are important to provide ongoing certainty of the clinical benefit for immunocompromised patients against new SARS-CoV-2 variants.

Anemia, Leukemia, and Retinal Findings

Hematological pathologies can cause retinal involvement along with all tissues of the body. Pathologies include many diseases such as anemia, leukemia, lymphoma, myeloproliferative and myelodysplastic. It is not uncommon for ocular findings to appear as the first symptom in these patients. Retinal involvement is most common in clinical practice. Since the symptoms are usually non-specific, it is difficult to diagnose. In this review, anemia and leukemias from hematological pathologies will be discussed and their effects on the retina will be explained.

Some results of a pilot study of mutations in ASXL1 and DNMT3A genes in myelodysplastic syndrome

Оbjective : was to determine the prevalence of the c.1934dupG mutation in the ASXL1 gene and the R882H mutation in the DNMT3A gene in MDS and their influence on significant clinical characteristics. Materials and methods : 33 men and 17 women with a median age of 57 years (18 –83) and a verified diagnosis of myelodysplastic syndrome were included in the study. Twenty-two volunteers without hematologic pathology were taken as controls, of them: 8 men and 14 women aged from 22 to 65 years. PCR examination of venous blood was performed in all study groups to detect c.1934dupG and R882H with Sanger reference sequencing. Results : the R882H mutation was not detected in any of the study groups. The c.1934dupG mutation was not detected in individuals without hematologic pathology. Due to a lack of concentration of isolated DNA from venous blood cells, no analysis of amplification efficiency could be performed in 7 patients. The c.1934dupG mutation was found in 46% of patients and was found in all IPSS-R, WPSS, and MDS-CI risk groups. No differences were found in the analysis of survival in the presence and absence of the c.1934dupG mutation. Conclusions : the study demonstrated that the developed method for the detection of the c.1934dupG mutation in venous blood cells enables optimization of diagnosis. A limitation of the adapted amplification and restriction assays was the degree of blood leucopenia. No effect of the c.1934dupG mutation on the clinical course of myelodysplastic syndrome was detected.

Effects of Replacement Cold Press Poppy Seed (Papaver somniferum) Oil to Fish Oil at Different Proportions on the Growth Performance, Blood Parameters, and Digestive Tracks Histopathology in Juvenile Common Carp (Cyprinus carpio)

The major goal of the study is to determine the potential use of poppy seed oil as a main lipid source in common carp diets. To investigate this, five different experimental diets (PO0, PO25, PO50, PO75, and PO100) with various proportions of fish oil and poppy seed oil blended as oil sources were provided and supplied to common carp for 90 days. In triplicate groups of 60 fish, 300 common carp weighing 1.15 ± 0.06 g were randomly placed in 100 L aquariums. At the end of the feeding trial, blood and tissue samples were collected from the fish after gravimetric measurements of the fish. The growth and feed utilization performance of the fish were calculated by gravimetric measurements, and tissue samples were examined by hematological, micromorphometric, and histopathological methods. According to the results, poppy seed oil enhanced the quantity of linoleic acid (18 : 2 ω−6) in the diet, and fish fed with the diet consisting completely of poppy seed oil (PO100) had the significantly greatest growth and feed utilization performance ( P < 0.05 ). Furthermore, no significant differences in hematological or liver pathology were detected between all groups that were fed with experimental diets ( P > 0.05 ). However, as compared to the control group, the digestive system micromorphometry and histomorphometric measurements of the fish fed with PO100 group diets showed a significant increase ( P < 0.05 ). In conclusion, it was determined that the carp fed with feeds produced by using only poppy oil as the main fat source improved their digestive system, growth, and feed utilization performance without any negative effects.

Changes in Coagulation Study and Risk of Developing Cholesteatoma: Is There a Link?

The etiopathogenesis of acquired pediatric cholesteatoma has not yet been fully clarified. Recent studies and modern technologies have led researchers to look for explanations at a molecular level. This study aims to understand if the origins of cholesteatoma could be related to dysfunctions in coagulation factors, thereby emphasizing its role in angiogenesis. Subjects and. This was a retrospective case-control study carried out at a tertiary hospital center between January 2010 and December 2020. The study included 92 children. The variables of the summary coagulation study (partial thromboplastin time, prothrombin time, and international normalized ratio) were compared among children with and without development of chronic otitis media with cholesteatoma. The cases and controls were comparable in terms of age, type, and number of times that ventilation tubes were placed. Partial thromboplastin times tended to be higher in children who developed cholesteatoma, with a statistically significant difference between the two groups in terms of normal and abnormal partial thromboplastin times (p=0.029). The results of this case control study indicate that slight extension of partial thromboplastin times in the coagulation study may not meet the criteria for diagnosis of certain hematological pathologies or clinical significance, but at a molecular level may already have implications for activation of angiogenesis and other growth factors involved in the onset, growth, and expansion of acquired pediatric cholesteatoma.

Laboratory Markers of Platelet Production and Turnover

Platelets are formed from bone marrow megakaryocytes, circulate in blood for 7-10 days, and then are destroyed in the spleen and/or liver. Platelet production depends on the megakaryocyte population state in the bone marrow: number and size of the cells. The platelet turnover, i.e., the number of platelets passing through the bloodstream in a certain time, is determined by both the rate of their production and the rate of their destruction. The review considers laboratory markers, which are used to assess platelet production and turnover in the patients with hematologic and cardiovascular pathologies. These markers include some characteristics of platelets themselves: (i) content of reticulated ("young") forms in the blood detected by their staining with RNA dyes; (ii) indicators of the platelet size determined in hematology analyzers (mean volume, percentage of large forms) and in flow cytometers (light scattering level). Alterations of platelet production and turnover lead to the changes in blood plasma concentrations of such molecules as thrombopoietin (TPO, main mediator of megakaryocyte maturation and platelet formation in the bone marrow) and glycocalicin (soluble fragment of the membrane glycoprotein Ib detached from the surface of platelets during their destruction). Specific changes in the markers of platelet production and turnover have been observed in: (i) hypoproductive thrombocytopenias caused by suppression of megakaryocytes in the bone marrow; (ii) immune thrombocytopenias caused by accelerated clearance of the autoantibody-sensitized platelets; and (iii) thrombocytosis (both primary and reactive). The paper presents the data indicating that in patients with cardiovascular diseases an increased platelet turnover and changes in the corresponding markers (platelet size indexes and content of reticulated forms) are associated with the decreased efficacy of antiplatelet drugs and increased risk of thrombotic events, myocardial infarction, and unstable angina (acute coronary syndrome).

Вроджені нейтропенії в дітей: короткий і довгий шлях до діагнозу

The diagnosis of neutropenia is based, first of all, on the results of the assessment of the absolute number of neutrophils in the complete blood count and the clinical picture. Genetic confirmation of the diagnosis of congenital neutropenia can be long and not always successful. The purpose of work - to analyze the clinical course and laboratory features of congenital neutropenia in children on bases of two clinical cases in order to pay the doctors’ attention to the diagnosis of these diseases. We present clinical cases that confirm the variability of clinical and laboratory manifestations and the severity of the course of neutropenia. In the first case, congenital neutropenia was manifested by profound clinical signs with cyclicity, was accompanied by fever, aphthous stomatitis, and gingivitis. The absolute neutrophil count was consistent with severe neutropenia, but it took several years for genetic confirmation of the diagnosis. The second case of cytopenia, including neutropenia in a boy, was found in twins by chance, as the children had no clinical manifestations associated with neutropenia. Despite the obviousness of neutropenia in the general blood test with or without clinical manifestations, the path to a complete diagnosis is not always simple. Cytopenia in children, including neutropenia, requires a multidisciplinary approach to diagnosis, excluding both the causes of secondary neutropenia, hematological pathology, and immune disorders, primarily the phagocytic defects. Genetic testing can help verify an accurate diagnosis in patients with inborn errors of immunity, however, the genetic variants of a large number of congenital neutropenias remain unknown. Isolated neutropenia in children, as well as in combination with other cytopenias, require careful monitoring and the selection of optimal management tactics, as they can have serious complications. Following of patients will make it possible to expand knowledge about the disease course, the dynamics of symptoms, the occurrence of complications in order to prevent them. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institutions. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

HEMATOLOGICAL PARAMETERS IN THE MODEL OF IRON DEFICIENCY AND HEMATOLOGICAL ANEMIA

According to the World Health Organization, the most common (up to 90% of cases) hematological pathology is anemia, a condition characterized by a decrease in the level of hemoglobin in the blood due to a decrease in the total number of red blood cells or a violation of hemoglobin synthesis, which can have serious consequences for the body, such as decreased immunity, impaired respiratory and metabolic processes. Currently, it is necessary to improve the methods of prevention and treatment of anemia. To test new drugs for these purposes, it is necessary to create new models, including on laboratory animals. The aim of the study was to develop experimental models of iron deficiency (IDA) and hemolytic anemia (HA) on laboratory animals. The objects of the study were sexually mature male laboratory rats of the Wistar breed, aged 7 months and weighing 260 ± 20 g. 38 animals were involved in the experiment (for IDA n= 24, for HA n= 14). Laboratory animals were kept in the same external conditions for 3-4 individuals in a cage. To simulate IDA, rats were subcutaneously injected with the drug “Desferal”. To simulate GA, rats were intraperitoneally injected with the drug “Hydrochloric acid phenylhydrozine”. At each stage, blood was taken by cutting off the tip of the tail. A detailed blood test was performed on a hematological analyzer “Heska Element HT5”. As a result, it was found that when the drug “Desferal” was administered at concentrations of 125 and 250 mg / 100g per animal weight, hematological changes were observed in rats, such as an increase in red blood cells, platelets, leukocytes by stage 2 and a decrease to control values by stage 4. When the drug “Hydrochloric acid phenylhydrozine” was administered, hematological changes were detected in rats only within the experimental group. There was an increase in the indicators of erythrocytes, platelets, leukocytes by stages 2 and 3 and a decrease to control values by stage 4. Thus, when the studied drugs were administered in the presented dosages in vivo, a change in the hematological parameters of the blood was detected in the direction of an increase in their number due to adaptation to the effects of substances.

Germline RUNX1 variants in paediatric patients in a French specialised centre.

Familial platelet disorder with associated myeloid malignancy (FPD-MM; OMIM 601399) is related to germline RUNX1 mutation. The pathogenicity of RUNX1 variants was initially linked to FPD-MM phenotype, but the discovery of new variants through the expansion of genetic explorations in leukaemia is questioning this assertion. In this study, we add 10 families with germline RUNX1 variant explored at Armand Trousseau Hospital for leukaemia diagnosis or thrombocytopenia, to the 259 described so far. Detailed description of their personal and family history of haematological pathologies allows identifying three phenotypes related to germline RUNX1 variants: thrombocytopenia and/or malignant haematological disease with family history of haematological diseases, thrombocytopenia with no family history of haematological diseases and acute lymphoblastic leukaemia (ALL) with no family history of haematological diseases. In the latter phenotype, ALL characteristics involving RUNX1 suggest the implication of germline variants in the onset of the malignancy.

An Update on the Current and Emerging Use of Thiazolidinediones for Type 2 Diabetes.

Guidelines have increasingly stressed the concept that adequate glycemic control is required to prevent or decrease the macro- and microvascular complications of type 2 diabetes mellitus (T2DM). PPAR-gamma agonists (“glitazones”) are no longer prioritized due to their effects on heart failure. However, the association between these drugs and innovative therapies could be a valuable tool to attenuate the risk factors of the metabolic syndrome. Glitazones are used for the treatment of diabetes and associated comorbidities. There is substantial scientific evidence demonstrating the effect of glitazones at a cardiometabolic level, as well as on hematological and neurological pathologies that point to their usefulness. The use of glitazones has always been controversial both for the type of patients who must take these drugs and for the side effects associated with them. Unfortunately, the recent guidelines do not include them among the preferred drugs for the treatment of hyperglycemia and rosiglitazone is out of the market in many countries due to an adverse cardiovascular risk profile. Even though real-life studies have proven otherwise, and their pleiotropic effects have been highlighted, they have been unable to achieve primacy in the choice of antihyperglycemic drugs. It would be appropriate to demonstrate the usefulness of pioglitazone and its therapeutic benefit with further cardiovascular safety studies.

Ecotoxicological studies of surface and groundwater in the adjacent territories of the Shuakhevi HPP (Adjara AR)

In the pathogenesis of autoimmune diseases, including immune thrombocytopenia (ITP), maintenance of inflammation and the loss of immune tolerance is essential. Transforming growth factor-beta (TGF-β) is a regulatory cytokine with pleiotropic function. TGF-β inhibits differentiation of T helper (Th1, Th2) cells and promotes the development of peripheral Tregs. The aim of our study was to explore the plasma levels of TGF-β and to perform its correlative analyses with peripheral blood biomarkers of systemic inflammation (NLR, PLR, PMR, SII, dNLR) in patients with ITP who did not respond to the first line treatment and had splenectomy as a second line therapy. The concentrations of TGF-β in plasma was quantified using ELISA kits by eBioscience. Statistical analyses was performed using Graph Pad Prism - Mann Whitney U test; for the correlation analyses we used spearman rank correlation by SPSS program. Our study revealed that the level of TGF-β in patients with refractory ITP is significantly diminished compared to the healthy subjects or patients undergoing splenectomy for other reasons than autoimmune or malignant hematological pathologies. There was no correlation observed between the plasma concentrations of TGF-β and the peripheral blood biomarkers of systemic inflammation.

Cryptogenic stroke and extrasystolic arrythmia: is there any correlation?

Abstract Up to 45% of all ischemic strokes (IS) are cryptogenic. Purpose To reveal the possible reasons of cryptogenic IS. Methods We analyzed the cases of computer tomography verified IS in patients admitted the hospital during the period of 3 years. All these 268 patients had unidentified source of IS. We analyzed hemostasiograms, lipids levels, performed echocardiography, Doppler ultrasound of brachiocephalic arteries, 24-hours ECG monitoring and common carotid arteries sphygmography (SG). Results None of the patients had atrial fibrillation, hemodynamically significant stenoses of brachiocephalic arteries or hematological pathology associated with hypercoagulation. 234 (87.3%) patients had atherosclerotic plaques in carotid area bifurcation (in 36 (13.4%) – intima-media thickness was 1.1 mm, in 74 (27.6%)– stenosis <40%, in 124 (46.3%) had 40–69% stenosis in diameter). The internal carotid stenosis in diameter is calculated by ECST, NASCET and St. Mary's ratio criteria. To detect the type of the plaques we used Gray-Weale–Nicolaides ultrasound classification. In 39 (14.5%) was type I: uniformly echolucent plaque; in 88 (32.8%) type II: predominately echolucent plaque; in 71 (26.5%) type III: predominantly echogenic plaque, none had type IV: uniformly echogenic plaque or type V: heavy calcification. In 24-hours ECG monitoring and SG we revealed that most of the patients – 244 (91.0%) had extrasystolic arrhythmia which ventricular contraction of extrasystole appeared in the early phase of cardiocycle – before the transmitral blood flow peak. In 1st post-extrasystolic wave after such extrasystole we detected the growth of linear blood flow speed in carotid bifurcation (2.5 m/sec) and kinetic arteries parameters of SG (2.6 times). Conclusion The appearance of IS is higher in patients with extrasystolic arrhythmia appearing in the cardiocycle before the transmitral blood flow peak. In cryptogenic stroke, the reason of it can be the atherosclerotic plaque defragmentation with further embolism, caused by the additional mechanical trauma of increased hemodynamical and kinetic parameters of pulse wave of the 1st post-extrasystolic contraction. Funding Acknowledgement Type of funding sources: None.

Ecotoxicological studies of surface and groundwater in the adjacent territories of the Shuakhevi HPP (Adjara AR)

In the pathogenesis of autoimmune diseases, including immune thrombocytopenia (ITP), maintenance of inflammation and the loss of immune tolerance is essential. Transforming growth factor-beta (TGF-β) is a regulatory cytokine with pleiotropic function. TGF-β inhibits differentiation of T helper (Th1, Th2) cells and promotes the development of peripheral Tregs. The aim of our study was to explore the plasma levels of TGF-β and to perform its correlative analyses with peripheral blood biomarkers of systemic inflammation (NLR, PLR, PMR, SII, dNLR) in patients with ITP who did not respond to the first line treatment and had splenectomy as a second line therapy. The concentrations of TGF-β in plasma was quantified using ELISA kits by eBioscience. Statistical analyses was performed using Graph Pad Prism - Mann Whitney U test; for the correlation analyses we used spearman rank correlation by SPSS program. Our study revealed that the level of TGF-β in patients with refractory ITP is significantly diminished compared to the healthy subjects or patients undergoing splenectomy for other reasons than autoimmune or malignant hematological pathologies. There was no correlation observed between the plasma concentrations of TGF-β and the peripheral blood biomarkers of systemic inflammation.

Ghosal hematodiaphyseal dysplasia (GHDD) diagnosis and treatment: case report

Ghosal hematodiaphyseal dysplasia syndrome (GHDD) is a rare authosomal ressesive disorder characterized by increased bone density and regenerative corticosteroid-sensitive anemia.We describe GHDD in an 11-year old Azerbaijani boy with refractory anemia,mild thrombocytopenia and radiological metadiaphyseal dysplasia.The diagnosis was made based on clinical and laboratory examinations and genetic analysis.We have observed a significant improvement of anemia after administration of steroids. An 11-year-old boy with long-standing anemia, complained of fatigue,delayed physical development,and limited range of motion in the joint.Physical examination did not reveal LAP and hepatosplenomegaly.Among the dysmorphic craniofacial changes mentioned in the literature, has a tower-shaped skull,micrognotia,drooping ears,a long and wide philtrum,and a thin upper lip.Skeletal X-ray imaging showed fibrotic changes and varying degrees of osteopenia in the metaphysis of the long tubular bones. The blood count: Hb 7.0 g/dl,HCT 24.5%,reticulocytes 5.6%,MCV 78fL,MCHC 28.6 g/dl,WBC count 6860/mm3,platelets 165000/mm3,ESR 75 mm/h,anisocytosis in erythrocytes and platelets were observed in a peripheral blood smear.Hemoglobin electrophoresis,iron studies,vitamin B12 and folic acid were normal.Coombs test was negative.Bone marrow examination showed hypoplasia in erythroid and megakaryocytic series and dysgranulocytopoiesis. After detection of exon 12 ((p.Gly473Trp),rs149988492,CM215867) in the genetic panel analysis of anemia,steroid treatment at a dose of 1 mg/kg/day was started and anemia improved at 1-month follow-up (Hb level 6.8 g/dL to 11.9 g/dL),but mild thrombocytopenia was noted to persist.The clinically insignificant CRP elevation normalized during the treatment. GHDD should be considered in patients with clinical and radiographic evidence of diaphyseal dysplasia as well as hematological abnormalities. In addition, bone dysplasia should be investigated in treatment-resistant hematological pathologies of unknown origin. Although GHDD is rare, clinicians should be informed that it responds well to steroid therapy.

Factors associated with bone marrow involvement in lymphoma staging bone marrow examination: A South African single-centre retrospective study

Background: Accurate detection of bone marrow involvement (BMI) in lymphoma is important as it signifies stage 4 disease. Staging bone marrow examination (BME), therefore, influences treatment decisions and prognostication. The prevalence of BMI depends on multiple factors at presentation including lymphoma subtype, age, sex, human immunodeficiency virus (HIV) status and haematological parameters.Aim: To determine risk factors for BMI in lymphoma staging.Setting: The study was conducted in the department of haematological pathology, Tygerberg Hospital, Cape Town, South Africa.Methods: Retrospective cross-sectional descriptive study in adult patients, reviewing BMI and associated parameters, during their initial lymphoma staging procedure between 2016 and 2019.Results: Of a total of 387 lymphoma staging cases that were evaluated, 30.0% of them showed BMI. Diffuse large B-cell lymphoma, Hodgkin lymphoma and high-grade B-cell lymphoma were the most frequent subtypes diagnosed. The highest prevalence of BMI was in low-grade lymphomas. There was a statistically significant association between BMI and advanced age, pancytopenia and bicytopenia (anaemia with leucopenia, anaemia with thrombocytopenia or leucopenia with thrombocytopenia). Bicytopenia and pancytopenia showed high positive predictive values of BMI, respectively, 61.0% and 69.0%. Human immunodeficiency virus positivity (34.6%) was not predictive of BMI across all lymphoma subtypes. Normal blood counts had a high negative predictive value for BMI.Conclusion: BME remains an important part of lymphoma staging with 30.0% of all lymphomas showing BMI.