11 research outputs found

    RARE DISEASES OF THE BLOOD SYSTEM: A CLINICAL CASE OF VIRUS-ASSOCIATED BONE MARROW APLASIA IN A PATIENT WITH A HEREDITARY MEMBRANOPATHY (REVIEW)

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    The aim of study was to research and present literature data regarding the investigations of some combined types of anemia in pregnancy and to describe the clinical case of pregnancy associated with primary parvovirus B19 infection and newly diagnosed hereditary erythrocyte membrane disorder. The contemporary information about the etiology, the prevalence, structure and some features of pathogenesis of parvovirus infection based on literature data was given. The huge prevalence of this infection (according to data of different sources the serum level of parvovirus B19 specific antibodies may be detect in 90 % of population), its threat for fetus and risk of severe complications in patients with immunodeficiency, as well as possibility of development of total depression of hematopoiesis in patients with hereditary erythrocyte membrane disorders, such as Minkowsky-Chauffard disease, determine the relevance and significance of the discussed topic. Furthermore, this clinical case, which illustrates the features of primary parvovirus B19 infection in patient with hereditary hemolytic anemia and the difficulties of differential diagnosis, demonstrates the importance of parvovirus infection markers detection, which is not available in the most TORCH-panel

    Prognostic value of the modified multiple myeloma comorbidity index in real clinical practice

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    Background. An increase in the number of patients with multiple myeloma (MM) necessitates the creation of reliable tools for assessing their somatic status (comorbidity) in order to personalize the optimal treatment regimen that helps to minimize its toxicity, improves survival and patients quality of life.The objective of this study was to modify the MM comorbidity index (MCI) by adding an additional variable reflecting the biological properties of the tumor, and to determine the informativeness of the new scale – a modified MM comorbidity index (M-MCI), to predict the outcome and select personalized therapy in patients with MM in real clinical practice.Materials and methods. From January 2012 to December 2017 the study included 369 patients with newly diagnosed MM (134 men and 235 women) who were hospitalized in the hematology department of the City Clinical Hospital No. 2, Novosibirsk. The median age of the patients was 67 (32–82) years. The prognostic value of concomitant diseases and individual prognostic factors in relation to the overall survival of patients with MM was evaluated.Results. Cox multivariate analysis showed that the most significant predictors of reduced overall survival of patients with MM are impaired renal function (glomerular filtration rate <30 ml / min / 1.73 m2 (according to the CKD-EPI formula), general condition according to the Karnowski scale ≤70 %, chronic obstructive pulmonary disease with moderate (50 % ≤ forced expiratory volume in 1 second <80 %) and severe (30 % ≤ forced expiratory volume in 1 second <50 %) severity of bronchial obstruction and the ratio κ / λ free light chains <0.04 or >65. These factors were combined into a weighted 5‑point scale M-MCI. A comparative analysis of survival depending on the value of the M-MCI allowed us to distribute patients with MM into groups of high (M-MCI 3–4 points) and standard (M-MCI 0–2 points) risk with significantly different indicators of overall survival (median overall survival amounted to 15.5 months in the high and 60 months in the standard risk group; χ2 = 58, p <0.016) and confirm the prognostic value of M-MCI in relation to the outcome of MM.Conclusion. In terms of its prognostic significance in predicting an adverse outcome, the proposed M-MCI scale is superior to its prototype – the MCI. The median overall survival in the high-risk group according to the M-MCI was 15.5 months compared to 20 months according to the MCI; the median overall survival in the group the standard risk was 60 and 50 months, respectively (χ2 = 58 (M-MCI) versus χ2 = 42 (MCI); p <0.001). The advantages of M-MCI are also its more accurate assessment of the physical condition of patients with MM and its simple clinical applicability

    The Profile of MicroRNA Expression in Bone Marrow in Non-Hodgkin’s Lymphomas

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    Non-Hodgkin’s lymphomas (NHLs) are a heterogeneous group of malignant lymphomas that can occur in both lymph nodes and extranodal sites. Bone marrow (BM) is the most common site of extranodal involvement in NHL. The objective of this study is to determine the unique profile of miRNA expression in BM affected by NHL, with the possibility of a differential diagnosis of NHL from reactive BM changes and acute leukemia (AL). A total of 180 cytological samples were obtained by sternal puncture and aspiration biopsy of BM from the posterior iliac spine. All the cases were patients before treatment initiation. The study groups were NHL cases (n = 59) and AL cases (acute lymphoblastic leukemia (n = 25) and acute myeloid leukemia (n = 49)); the control group consisted of patients with non-cancerous blood diseases (NCBDs) (n = 48). We demonstrated that expression levels of miRNA-124, miRNA-221, and miRNA-15a are statistically significantly downregulated, while the expression level of let-7a is statistically significantly upregulated more than 2-fold in BM in NHL compared to those in AL and NCBD. ROC analysis revealed that let-7a/miRNA-124 is a highly sensitive and specific biomarker for a differential diagnosis of BM changes in NHL from those in AL and NCBD. Therefore, we conclude that analysis of miRNA expression levels may be a promising tool for early diagnosis of NHL

    ADULT-ONSET STILL’S DISEASE: ASPECTS OF THE HEMATOLOGY CLINIC

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    The analysis of literature data on modern approaches in the diagnosis and treatment of adult-onset Still’s disease has been performed. A clinical case of Still’s disease associated with lymphadenopathy syndrome was demonstrated. It is noted that the diagnosis of adult Still’s disease requires a doctor to exclude a complex of a tumor, rheumatological and infectious pathology. It is important for a hematologist not to miss a blood tumor and to prevent a diagnostic error. The authors showed the criteria for exclusion of myeloblastic hematosarcoma and malignant lymphoma in a patient with Still’s disease. The importance of taking into account the clinical data is noted: the absence of progressive sarcomal growth of lymph nodes, the lack of generalization and dissemination of myeloblastic substrate in the body, the reduction of fever with steroids and nonsteroidal anti-inflammatory drugs. The role of monitoring clinical and biochemical blood parameters has been proved such as: blood ferritin level, C-reactive protein, hemogram parameters. The relative value of positron emission computed tomography data, which can be falsely interpreted, is shown. Correct diagnosis, rejection of an incorrect diagnosis of hematological tumor will prevent the administration of myeloablative polychemotherapy, which is dangerous for an adult patient with Still’s disease. The article outlines the differential diagnosis algorithm, presents the criteria for diagnosis and approaches to therapy. A feature of the clinical case was the detection of antibodies against yersenia. A meeting with an infectious agent could be a trigger for the development of a multisystem form of Still’s disease in a patient
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