[Detection of paraprotein in plasma cell tumors].

Paraprotein is a laboratory biomarker of plasma cell tumors and other lymphoproliferative diseases. Its determination is necessary for diagnosing, monitoring and assessment of therapy effectiveness. The lecture presents the main methods of qualitative and quantative analysis of monoclonal proteins: gel electrophoresis, capillary electrophoresis, immunofixation and nephelometry features, possibilities and limitations are reviewed. The main sources of errors and artifacts during these studies are considered. Also the difficulties in the diagnosis and interpretation of the results of serum and urine tests are highlighted.

[Autologous haematopoietic stem cell transplantation in patients with multiple myeloma complicated by dialysis-dependent renal failure].

AIM: To assess the safety and efficacy of autologous haematopoietic stem cell transplantation (auto-HSCT) in multiple myeloma (MM) patients with dialysis-dependent renal failure. MATERIALS AND METHODS: During a period from May 2010 to December 2016 fourteen MM patients with dialysis-dependent renal failure aged 48 to 65 years underwent auto-HSCT. After the induction therapy complete response, very good partial response, partial response were documented in 64, 29, 7% of patients, respectively. In no case was a renal response achieved. Haematopoietic stem cell mobilization in most patients (13/14) was performed according to the scheme: G-CSF 10 g/kg. Melphalan in 3 dosages was used as pre-transplant conditioning: 100, 140 and 200 mg/m2; 13 patients underwent a single and in one case underwent a tandem auto-HSCT against the background of hemodialysis. Evaluation of the antitumor and renal response was assessed on the 100th day after auto-HSCT. Subsequently, against the background of programmed hemodialysis and in the setting of high-dosed melphalan (100200 mg/m2), 13 patients underwent a single and one patient underwent a tandem auto-HSCT. At +100 days after auto-HSCT, an antitumor response and renal response were assessed. RESULTS: The period of agranulocytosis after auto-HSCT was from 5 to 12 days (median 8,5) and was accompanied by infectious complications, cardiac and neurological dysfunctions. At +100 days after auto-HSCT, the complete response was confirmed in 71% patients and very good partial response was confirmed in 29% patients. The minimal renal response was registered in 2 patients (14%), hemodialysis was stopped. The transplant-related mortality was absent. After a median follow-up of 53 months 5-year progression-free survival was 59%, and overall survival was 93%. CONCLUSION: Carrying out auto-HSCT in patients with dialysis-dependent renal failure contributed to the achievement of a minimal renal response in 14% of cases, which allowed these patients to stop hemodialysis. Patients whose conditioning regimen was performed using melphalan at a dose of 200 mg/m2showed more frequent complications in the early post-transplant period compared to patients who received a lower dose of melphalan (100140 mg/m2). Auto-HSCT in MM patients with dialysis-dependent renal failure is a feasible and effective treatment method, which in some cases contributes to independence from hemodialysis.

[Difficulties evaluating hematological response in patients with multiple myeloma and dialysis - dependent renal impairment].

AIM: to determine serum free light chains (FLC) level by patients with multiple myeloma (MM) and dialysis - dependent renal impairment in which the amount Bence Jones (BJ) protein in the urine met the criteria of hematological response. PATIENTS AND METHODS: This study included 13 MM with dialysis - dependent renal impairment patients (estimated glomerular filtration rate < 10 ml/min), whose urine BJ protein content was less than 200 mg/day after antimyeloma therapy (including 11 patients whose urine BJ protein content was less than 100 mg/day). RESULTS: The median serum concentration of monoclonal FLC was 608.7 (298-8380) mg/l. Thus, with trace amounts BJ protein in the urine serum content monoclonal FLC varied 28 times with the same degree of severity of renal failure. In patients with oliguria serum SLC content was significantly higher than in normal diuresis (1109 and 307 mg/L; p.

Immunoglobulinopathies in patients with angioimmunoblastic T-cell lymphoma.

AIM: The aim of the study was to characterize quantitative and qualitative immunoglobulinopathies in patients with AITL at the onset of the disease. MATERIALS AND METHODS: 55 patients with newly diagnosed AITL were enrolled in the study, the male/female ratio was 30/25; median age was 61 (29-81) years. Diagnosis was based on standard WHO criteria. Immunochemical studies of blood serum included serum protein electrophoresis/immunofixation, nephelometric quantification of total immunoglobulins, serum free light chain assay. RESULTS: Quantitative and qualitative immunoglobulinopathies were determined in 49 (89,1%) of 55 pts. Quantitative immunoglobulinopathies were revealed in 47 (85.5%) of 55 cases, qualitative - in 14 (25,5%). Combination quantitative and qualitative immunoglobulinopathies was observed in 12 (21,8%) of 55 pts. The detected immunoglobulinopathies were divided into 4 groups: polyclonal hypergammaglobulinaemia, hypogammaglobulinaemia, oligoclonal gammapathy, and monoclonal gammapathy. Polyclonal hypergammaglobulinaemia was marked in 41 (74.5%) of 55 pts, elevated level of IgG was determined in 27 (49,15%) of 55 cases, IgM - in 18 (32,7%) and IgA - in 21 (38.2%). Interestingly, polyclonal IgE hypergammaglobulinaemia was detected in 12 (48,0%) of 25 cases of performed studies. Hypogammaglobulinaemia was detected in 8 (14,5%) of 55 cases. Oligoclonal gammapathy was determined in 4 (7.3%) of 55 pts. Monoclonal gammapathy was revealed in 11 (20,0%) of 55 cases. The amount of monoclonal immunoglobulin varied from 2.6 to 14.1 g/l. Monoclonal immunoglobulin Gk was detected in 5 of 11 pts, Gλ - in 2, Mλ - in 2, Mk - in 2. Monoclonal gammapathy was accompanied by polyclonal hypergammaglobulinaemia in 9 of 11 cases, hypogammaglobulinaemia - in 2. CONCLUSION: Quantitative and qualitative immunoglobulinopathies are observed in most patients at the onset of AITL. Quantitative abnormalities were determined more often than qualitative. Monoclonal gammapathy can be a manifestation of lymphoproliferation and other concomitant disorders. The prognostic value of immunochemical parameters is still unclear and requires dynamic observation and study.

[Hereditary afibrinogenemia: A literature review and clinical observations]. / Nasledstvennaia afibrinogenemiia: obzor literatury i klinicheskie nabliudeniia.

Afibrinogenemia is a rare congenital coagulopathy that leads to life-threatening bleeding. In afibrinogenemia, plasma fibrinogen levels are less than 0.1 g/L. The clinical manifestations of the disease can be both bleeding and thromboses of different localizations, which is determined by the multifunctional role of fibrinogen in hemostasis. The described cases demonstrate different clinical phenotypes of the disease. In both cases the diagnosis was confirmed by genetic examinations that revealed homozygous mutations in the fibrinogen A genes. The nature of the mutations assumes consanguineous marriages, as confirmed by the results of a genealogical analysis. Fibrinogen preparations are promising in treating afibrinogenemia in Russia.

COMPARATIVE ANALYSIS OF SEROLOGICAL MARKERS OF HERPES VIRUSES AND QUANTITATIVE IMMUNOGLOBULINOPATHIES IN PRIMARY PATIENTS WITH ANGIOIMMUNOBLASTIC T-CELL LYMPHOMA.

INTRODUCTION: Angioimmunoblastic T-cell lymphoma (AITL) is associated with the Epstein-Barr virus (EBV) in most cases. It is believed polyclonal hypergammaglobulinaemia observed in 53-80% of AITL patients has anti-herpes viral antibodies as its substrate. AIM: The aim of the study was to compare serological markers of herpes viruses and quantitative immunoglobulinopathies of classes M and G in primary patients with AITL. MATERIALS AND METHODS: 26 primary patients with newly diagnosed AITL treated at the National Research Center for Hematology from 2002 to 2017 were enrolled in the study. The male/female ratio was 16/10; median age was 62 (29-81) years. The levels of total immunoglobulins of classes M and G, serological markers of EBV, cytomegalovirus (CMV) and herpes simplex virus type 1 and type 2 (HSV 1, 2) were assessed in all patients. RESULTS: Significant relationship was found between the presence of virus-specific IgM (IgM HSV 1, 2, IgM CMV, IgM VCA EBV) and an elevated level of total immunoglobulins of class M (p.

[Renal lesion in hemoblastoses].

AIM: To study morphofunctional condition of the kidneys in patients with hematological malignancies. MATERIAL AND METHODS: Renal function, i.e. concentration ability, glomerularfiltration rate (GFR), concentration of electrolytes and beta2-microglobulin in blood serum and urine was studied at different stages of chemotherapy. Three cases are reported. All the patients have undergone transcutaneous fine-needle puncture biopsy of the kidney with histological and electron-microscopic study of biopsy specimens. RESULTS: Total urinalysis showed minimal changes. Concentration and filtration functions of the kidneys were impaired. Morphologically, glomerular damage was insignificant, there was dystrophy of tubular epithelium and focal interstitial sclerosis. Electron-microscopic examination revealed structural defects of the basal membrane of glomerular capillaries (focal thinning, focal insignificant thickening, the absence of three-layer structure at long distance) in all the patients. Dissection of the basal membrane was seen in one case. All the patients had cytoplasmic viruses: Epstein-Barr, cytomegalovirus, herpes simplex viruses type 1 and 2. In two cases mitochondria were affected with viruses. Dystrophic changes of podocytes and mitochondrial degradation were observed in all the cases. CONCLUSION: Chemotherapy may affect glomerular and tubular system of the kidney increasing probability of complications. Imbalance between cytolysis kinetics and natural clearance of the products of degradation of cells and drug metabolites may lead to nephropathy. Viral infection indicates immunodeficiency due to immunosuppression or is a direct cause of impairment of renal endothelium cells. No definite recommendations can be made still about therapeutic policy.

[Cardiac contractile function in the participants of the cleanup of the aftermath of the accident at the Chernobyl Atomic Electric Power Station who are ill with hypertension]. / Sokratitel'naia funktsiia serdtsa u uchastnikov likvidatsii posledstvii avarii na ChAES, bol'nykh gipertonicheskoi bolezn'iu.

In a major proportion (67%) of those examined (persons with stage-II hypertensive disease who took part in the elimination of the aftermath of the Chernobyl breakdown) with the aid of echocardiography, hypertrophy of the left ventricular (LV myocardium was revealed as was augmentation of the mass of the LV myocardium together with decline in the contractile function, while in other examinees (33%) all above parameters showed no deviation from the norm. This permits predicting probability of cardial complications in such patient populations.

[Hyperbaric oxygenation in the treatment of patients with vibration disease due to local exposure]. / Giperbaricheskaia oksigenatsiia v lechenii bol'nykh vibratsionnoi bolezn'iu, obuslovlennoi lokal'nym vozdeistviem.

Results are reported of treatment of 69 patients with vibration disease (VB) using hyperbaric oxygenation. Treatment course--10 sessions with an exposition of 40 minutes in the main regimen. Tests for choice of treatment regimens (1.3, 1.5, 1.8 atm.) were the dynamics of subjective and objective clinical signs, indices of the peripheral hemodynamics and thermoregulation. Hyperbaric oxygen treatment in persons with signs of vibration effects (preclinical stage) using a working pressure of 1.3 atm is justified. Presence of clinical manifestations of the disease (grade I-II degree of VB severity) justifies increase of the dose of hyperbaric oxygen to 1.5 atm.

[Evaluation of the factors providing the oxygen regimen to tissues in miners with vibration disease receiving combined therapy with a course of hyperbaric oxygenation]. / Otsenka faktorov, obespecheniia kislorodnogo rezhima tkanei gornorabochikh, bol'nykh vibratsionnoi bolezn'iu, pri kompleksnoi terapii s kursom giperbaricheskoi oksigenatsii.

In 4 patients with vibration disease hyperbaric oxygenation was included in the complex treatment and this resulted in an increase of blood supply to the extremity, increase of vascular reactivity, reduction of the peripheral vascular resistance. Inverse dependence of the hyperbaric oxygenation treatment and severity of vibration disease was found that was mainly determined by the initial lever of the peripheral hemodynamics.

[Current status and trends in the health of the Moscow population]. / Sovremennoe sostaoinie i tendentsii zdorov'ia naseleniia Moskvy.

Based on vast comprehensive medical statistical database, the authors analyze the health status of the population and the efficacy of public health service in Moscow. The pre-crisis tendencies and the modern status of public health under modern socioeconomic conditions are noted.

[The significance of an alpine climate in the combined treatment of occupational diseases]. / Znachenie gornogo klimata v kompleksnoi terapii nekotorykh professional'nykh zabolevanii.

The authors studied the influence of mountain climate (altitude: 2200 m) on the course of chronic dust-induced bronchitis, pneumoconiosis and vibration disease in 45 patients. It was found that a 1-month stay of patients with dust-induced bronchitis and pneumoconiosis in the mountains results in a positive clinical effect is accompanied by amelioration of the external respiratory function, positive changes of the hemodynamic values. Mountain climate treatment was ineffective in vibration disease.

[The results of the combined treatment of patients with dust-induced bronchitis]. / O rezul'tatakh kompleksnogo lecheniia bol'nykh pylevym bronkhitom.

A study in 84 patients with dust-induced bronchitis receiving combined drug treatment, hyperbaric oxygenation, hypobarotherapy and artificial speleotherapy indicates that the first place by its efficacy is occupied by artificial speleotherapy resulting in an improvement of the functional indices of the cardiopulmonary system. The second place is occupied by hypobaric regimen. Hyperbaric oxygenation was of low efficacy.

[The use of hyperbaric oxygenation for the treatment of patients with pneumoconiosis and pulmonary heart failure]. / Primenenie giperbaricheskoi oksigenatsii dlia lecheniia bol'nykh pnevmokoniozom s legochno-serdechnoi nedostatochnost'iu.

Examination of 76 patients with pneumoconiosis (stage I) revealed that hyperbaric oxygenation is increasing the force of cardiac contractions, reduction of the general peripheral resistance and reduction of the lesser circulation hypertension. The therapeutic action of hyperbaric oxygenation is related to its ability to remove the sequels of oxygen.

[Epidemiologic features of syphilis prevalence in Moscow]. / Epidemiologicheskie osobennosti rasprostraneniia sifilisa v Moskve.

The status of the dermatological and venereological service in Moscow is analyzed and the epidemic situation for syphilis described for the period of 1992-1996. The activities of the service are assessed and its prospects for the nearest future outlined.