[The specific features of pain syndrome in patients with hemophilia]. / Osobennosti bolevogo sindroma u patsientov s gemofiliei.

AIM: to study the nature of pain in hemophilic arthropathy and its prevention ways used by patients with hemophilia. SUBJECTS AND METHODS: A prospective, multicenter, cross-sectional study was conducted to interview 136 patients with hemophilia A or B. The survey included 32 points, including questions about drug and nondrug pain treatment, as well as questionnaires to assess the severity of pain and its impact on daily life (Brief Pain Inventory) and those to identify a neuropathic pain component (PainDetect questionnaire). RESULTS: 83 (75%) patients with hemophilia experienced acute pain associated with bleeding into the large joints; 44 (39%) patients had chronic pain that had lasted longer than six months; 33% assessed the moderate pain experienced in the past 24 hours as severe (more than 5-8 of the 10 scores). In addition to the above, only 32% of the respondents indicated that they had painless intervals within the last 24 hours. 75% of the hemophilia patients mentioned to have limited daily activities. 74% reported their partial or complete disability when pain occurred. 77% of the respondents pointed out that when having pain, they had experienced great difficulty walking. 55% of the hemophilia patients had sleep disorders because of pain. When acute pain occurred, only 91 (81%) respondents injected a factor VIII or IX preparation, 37% of the respondents used narcotic analgesics; 51% received different nonsteroidal anti-inflammatory drugs; 13% took paracetamol. Some patients indicated that they used alcohol and illegal narcotic drugs to relieve pain. Attention is drawn to the fact that the hemophilia patients very frequently took painkillers: 60% of the respondents used analgesics every month; of them 49% had them every week, 11% every day. Most patients (n=83 (74%)) stated that they treated pain (prescribed drugs) themselves; 49 (44%) patients held they were dissatisfied with their pain treatment. When the question as to how the analgesic you used could "remove' pain was asked, only 12 (10%) patients answered that this could fully relieve the pain; 31 (26%) patients told that the pain was not reduced even by half. The study has shown that a neuropathic pain component is very common in hemophilia patients (31%). CONCLUSION: Pain in patients with hemophilia is a serious problem that negatively affects their quality of life, including their working capacity. The prevention and treatment of pain in hemophilia patients should involve the following: organization of educational activities and pain services; adequate treatment of acute pain; detection and prevention of a neuropathic pain component.

[INTENSIVE CARE OF COMPLICATED ACUTE PORPHYRIA].

The experience of anesthesia and intensive care departments of Hematological center Russia for intensive care management in 13 patients with acute porphyria, from 1996 till 2013 was summarized ion this pape4: Main causes of life-threatening complications of acute porphyria and its frequency were revealed Advantages of mechanical lung ventilation in respiratory failure, algorithms of clinical nutrition, correction of water-electrolyte disorders were represented. Importance of kinesiotherapy in successful treatment in these category of patients was revealed. It is shown that the whole complex of intensive care methods with the specific pathogenetical therapy brings success in 84,6 % of patients.

[EMERGENCY TREATMENT OF BLEEDING IN PATIENTS TAKING WARFARIN].

Anticoagulant therapy with vitamin K antagonists (AVK) is an effective treatment and prevention of thrombosis. One of the major disadvantages of the AVK is a risk for serious bleeding. Prothrombin complex concentrates (PCC), fresh frozen plasma (FFP) and vitamin K1 are available for control of these situations. The experience of special team ofthe Scientific Center for Hematology was the basis for presented retrospective study. Three regimens of warfarin-related bleeding were compared: PCC+ VK for several bleeding, FFP+ VK for different clinical situations and VKfor light bleeding. PCC showed himself as effective and safe hemostatic agent. Transfusions of FFP were sometimes not effective, sometimes led to TACO. Supplementation of vitamin K1 for patients of I and II groups provided more stable control of hemostasis. In III group VK vas effective to stop bleeding. Two impotent sings for conclusion: necessary of laboratory monitoring, TEG first of all; individual balance of hemostasis base of bleeding or thrombotic risks.

[Analgesia in hemophiliac patients during orthopedic surgery].

AIM: To develop an effective and safe analgesic regimen (by minimizing the proportion of narcotic analgesics) in hemophiliac patients in the perioperative period during high-trauma surgeries (total knee and hip replacements). SUBJECTS AND METHODS: The prospective study included 24 patients aged 22 to 57 years (median age 38 years) with severe congenital hemophilia A (n = 22) and B (n = 2), who had undergone knee (n = 18) or hip (n = 6) replacements in the Hematology Research Center, Russian Ministry of Health of the Russian Federation, in 2013. Two analgesic regimens (a) paracetamol with trimeperidine; b) paracetamol and ketamine with trimeperidine) were used. To assess pain intensity, the authors used scoring scales: a visual analogue scale (VAS) and a numeric rating scale (NRS). The effect of paracetamol on hemostasis was evaluated on the basis of thromboelastogram and coagulogram readings. Possible paracetamol hepatotoxicity was assessed analyzing liver enzymes. RESULTS: An interview has shown that 66% of the hemophiliac patients regularly take analgesics for chronic pain syndrome, among them 29% use narcotic analgesics. It is difficult to achieve perioperative analgesia in these patients. The dosage of narcotics can be decreased (that of trimeperidine on an average from 80 to 45 mg/day) in the early postoperative period if non-narcotic analgesics, such as paracetamol 4 g/day, are incorporated into the analgesic regimen. Paracetamol promotes pain relief to moderate and mild (not more than 40-50 VAS scores and 3-4 NRS scores), without affecting hemostasis (the thromboelastogram readings differed statistically insignificantly; the coagulation index was 0.6-1.6) and without having a hepatotoxic effect. CONCLUSION: The systemic use of analgesics in hemophiliac patients considerably makes postoperative analgesia difficult. The use of paracetamol with trimeperidine within the first 24 hours after high-trauma surgeries in hemophiliac patients (even if they have hepatitis C) is rather effective and safe.

[The particularities of acute surgical diseases treatment of abdominal cavity organs in patients with haemophilia].

The experience of treatment of 366 patients with haemophilia who were urgently hospitalized in hеmatological Scientific Center over the last 10 years is presented in the article. There were 114 (31.1%) patients with acute diseases of abdominal cavity organs, 150 (41%) patients with bleeding from upper gastrointestinal tract, 102 (27.9%) patients with acute hematomas of retroperitoneal space. Urgent operations were performed in 48 (22.2%) patients who were hospitalized with clinical symptoms of acute abdomen syndrome. It was developed the criteria of diagnosis and choice of treatment tactic on the basis of the received results. Application of presented algorithms led to improve the quality of urgent surgical care to patients with haemophilia.

[Thrombocytopenia and postoperative analgesia].

OBJECTIVE: To develop effective and safe measures of postoperative multimodal analgesia (optimizing the use of narcotic analgesics) in surgical treatment of hematological patients with thrombocytopenia. DESIGN: A pilot and prospective comparative controlled study. METHODS: We studied 27 patients with acquired thrombocytopenia who underwent laparoscopic splenectomy. Three schemes of anesthesia were used: 1) Nefopam 20 mg/day + trimeperidin (n = 7); 2) Paracetamol 4 g/day + trimeperidin (n = 10); 3) paracetamol + trimeperidin + glucocorticosteroids (GC) (through treatment of the underlying disease) (n = 10). Analgesic properties and effect of each scheme were assessed according to a rating scale of pain NRS (10 points) and VAS (100 points) studied. Additionally we evaluated the effect of nefopam and paracetamol on the functional properties of platelets and hemostasis, platelet levels while monitoring and indicators of thromboelastogram (TEG). RESULTS: Application of paracetamol + trimeperidin accompanied with effectively reducing of postoperative pain (less than 5 points on the NRS). The level of postoperative pain was lower in patients who were treated with corticosteroids within the therapy of the underlying disease before surgery and who continued to receive it in postoperative period (2-3 points NRS). Nefopam use in the perioperative period is not only inferior to the analgesic effect of paracetamol, but also causes frequent side effects. Consumption of narcotic analgesic--trimeperidin when applying nefopam averaged 43 mg/day, the appointment of paracetamol--28 mg/day, using a combination of paracetamol + GC--20 mg/day. Thus, GC within the underlying disease treatment substantially reduces the need for opioid analgesics. A monitoring of the number of platelets and TEG did not shows negative effect of paracetamol and nefopam on platelet and plasma hemostasis. CONCLUSIONS: Nefopam and paracetamol may be used in patients with thrombocytopenia, as do not affect the hemostasis and platelet count. Application of postoperative analgesia scheme paracetamol + trimeperidin in patients receiving corticosteroids provides the maximum reduction of pain with the least consumption of narcotic analgesics.

[Pulmonary artery catheterization in patients with blood diseases].

PURPOSE OF THE STUDY: To analyze complications of the pulmonary artery catheterization in patients with blood diseases. MATERIALS AND METHODS: 93 cases of pulmonary artery catheterization in patients with blood diseases were included in the retrospective study. RESULTS: Indications for pulmonary artery catheterization were septic shock (78.5%) and acute respiratory failure (21.5%). In 31 cases (33.3%) pulmonary artery catheterization was performed in conditions of agranulocytosis and in 81 cases (87%) in conditions of thrombocytopenia (platelets median 43 x 109 per liter minimal 7 x 109, maximal 150 x 109 per liter). Patients received transfusions of platelets in case of thrombocytopenia less than 30 x 109 per liter. Early complications of pulmonary artery catheterization occurred in 5 patients with thrombocytopenia (5.4%), the complications was connected with bleeding (hematoma, bleeding from place of puncture, lung bleeding) and mechanical (arterial puncture, pneumothorax, hemothorax). Number of attempts of the central vein catheterization was risk factor the complications. The frequency of catheter-associated sepsis was 5.89 cases each 1000 catheter-days and the frequency of infections of the soft tissues was 9.78 cases each 1000 catheter-days. Catheter-associated infection complications occurred in cases of catheter use over 5 days. Catheter-associated sepsis occurred in 2 of 3 patients with agranulocytosis. Other complications included intermittent arrhythmias during catheter moving in the heart chambers (58), catheter balloon rupture (4), and thrombosis of catheter lumen (3). CONCLUSIONS: Pulmonary artery catheterization can be used in patients with blood diseases and first of all in cases of septic shock and acute respiratory failure. Alternative less invasive methods of monitoring should be used in patients with agranulocytosis.

[Lung artery catheterization in patients with blood diseases].

UNLABELLED: Purpose of the study was to analyze complications of the lung artery catheterization in patients with blood disease. MATERIALS AND METHODS: 93 cases of the lung artery catheterization in patients with blood disease were studied in the retrospective research. RESULTS: Indications for lung artery catheterization were septic shock (in 78.5% of cases) and acute respiratory failure with different etiology (in 21.5% of cases). In 31 cases (33.3%) lung artery catheterization was performed in patients with agranulocytosis and in 81 cases (87%) in patients with thrombocytopenia (platelets median was 44 10(9)/L, from 7 10(9)/L to 7 150 10(9)/L). If a thrombocytopenia was less than 30 10(9)/L the patients received transfusion of platelets concentrates. Early complications of the lung artery catheterization occurred in 5 patients with thrombocytopenia (5.4%). Character of the complications was hemorrhagic (haematoma, bleeding from place of puncture, lung bleeding) and mechanical (puncture of artery, pneumothorax, haemothorax). Number of attempts of central veins puncture was a risk factor for the complications. Frequency of catheter associated sepsis was 5.89 cases each 1000 catheter-days. Frequency of soft tissues infection in the area of catheterization was 9.78 cases each 1000 catheter-days. Catheter associated infections occurred in cases when catheter was used more than 5 days. 2 of 3 patients with catheter associated sepsis had agranulocytosis. Other complications included intermittent arrhythmias during catheter moving through heart chambers (58), rupture of catheter container during its use (4), thrombosis of the one lumen of catheter (3). CONCLUSIONS: Lung artery catheterization can be used in patients with blood disease and first of all in patients with septic shock and acute respiratory failure. In patients with agranulocytosis less invasive methods of monitoring are more advisable.

[Endogenous heparin-like syndrome: analysis of clinical observations].

One of the reasons for non-surgical bleeding is heparin-like syndrome (HLS), under which is understanded presence of heparin effect in the absence of it's exogenous application. The role of endogenous heparins perform glycosaminoglycans -- biologically active substances. HLS is accompanied by endothelium damage and discussed in the network of the systemic inflammatory response syndrome (SIRS). HLS is described in liver future, sepsis, pregnancy and a number of hemoblastosis. Hypocoagulation effect of endogenous heparin localizates in X coagulation factor. The main method of diagnosis - thromboelastography. The use of a specific heparin antidote - Protamine sulfate has not confirmed clinical efficacy. Priority direction in the therapy of - methods of "shunt hemostasis". In this paper, we present the analysis of observations of 4 patients with developed endogenous HLS. In 2 cases (combination of sepsis with hepatic failure in one patient and initial thrombophilia in other) HLS has been accompanied by massive bleeding (massive hemothoraxc with haemorrhagic shock, a massive intraoperative blood loss). For HLS relief in these cases was used prothrombine complex concentrate (PCC) (in the 1st case), recombinant VIIa factor (in the 2nd case). In other cases, HLS (in a patient with multiple myeloma and childbirth in the postpartum period), haemorrhagic syndrome was not so expressed, the treatment was carried out with FFP transfusion.

[Therapy of acute massive blood loss in a hemophilic patient: case report].

A case is reported of management of massive intraoperative blood loss in a male patient with severe hemophilia. Extirpation of hip pseudotumor with one-stage osteosynthesis with an intramedullary joint-pin in a 43 year old male patient was accompanied with 7.5 l blood loss. The infusion-transfusion therapy (ITT) contained transfusion media about 1/3 of the total volume, fresh-frozen plasma and erythrocyte-containing media were used 1:1. Infusion solutions consisted of balanced polyelectrolytic solutions, hydroxyethylated starches 130/0.4, hyperchaes. Intraoperative normovolemic hemodilution and reinfusion of wound blood were made (CellSaver). ITT target markers were standard hemodynamics control tests. Hemostasis monitoring was conducted with thromboelastography. Complex ITT based on modern principles of clinical transfusiology provided a complete and safe compensation of massive intraoperative blood loss in a patient with severe hemophilia.

[Nine-year experience in the treatment of patients with diffuse large B-cell lymphosarcoma].

AIM: To ascertain indications to standard (CHOP-21/R-CHOP-21) and intensive (mNHL-BFM-90) treatment in patients with diffuse large B-cell lymphosarcoma (DLBCL) with involvement of lymphoid organs. MATERIAL AND METHODS: The trial, performed from January 2002 to December 2010, enrolled 139 DLBCL patients with affected lymph nodes (LN), tonsils, spleen, bone marrow (BM). The diagnosis was made according to WHO criteria. The patients were examined according to the protocol of lymphoproliferative diseases. Biopsy material from all 139 patients was studied immunohistochemically on paraffin blocks (LN, tonsils, spleen, BM) using a wide panel of antibodies. The same examinations of BM were made in all 18 cases of BM involvement. Cytogenetic examination was performed in 106 patients: 48 standard cytogenetic tests, 139 - FISH for t (14;18) as well as rearrangement of locus 3q27. Patients with a poor prognosis (n = 86, 61.8%) received intensive therapy according to mNHL-BFM-90 program. The signs of a poor prognosis were the following: massive tumor (tumor size more than 7.5 cm), invasion into the adjacent organs or tissues, stage III-IV disease by Enn-Erbor, high concentration of LDG. Patients without a poor prognosis (n = 53, 38.2%) received standard treatment CHOP-21 (n = 28) or R-CHOP-21 (n = 25). RESULTS: A complete remission without recurrences was achieved in all 53 patients without signs of unfavourable prognosis (100%). Overall 5-year survival was 96%, 2 patients died in remission of other causes. Of 86 patients with a poor prognosis a complete remission was achieved in 64 (74.4%) patients. Overall and recurrence-free 5-year survival was 65 and 86%, respectively. CONCLUSION: Standard treatment provided long-term complete remission in all the patients without poor prognosis. Intensive (mNHL-BFM-90) treatment produced the best results in generalized lesion without BM involvement. Overall 5-year survival was 84% in these patients and 12% in patients with BM involvement.

[Acute abdominal syndrome in blood system diseases].

AIM: To define an optimal diagnostic and therapeutic algorithm when the acute abdominal syndrome occurs in hematological patients. MATERIALS AND METHODS: The results of 145 emergency surgeries made in 2006-2008 for acute abdominal syndrome were studied in patients with blood system diseases. RESULTS: Clinical manifestations of acute abdominal syndrome emerge in 1-1.4% of all the patients treated at the Hematology Research Center, Russian Academy of Medical Sciences. There is a need for surgery in 0.5-0.7% of all the patients admitted. In this group of patients, annual postoperative mortality is 12-16%. CONCLUSION: The routine algorithm for a diagnostic search in hematological patients with acute abdominal syndrome can lead to both hyperdiagnosis and unwarranted surgery, and incorrect choice of expectant policy as well.

[Thromboelastographic characteristics of different infusion therapy regimens in healthy bone marrow donors].

Infusion solutions are able to change the hemostatic system. Thromboelastography (TEG) is an integral technique to evaluate the hemostatic system. TEG was used to evaluate the effect of three infusion solutions (6% hydroxyexyethyl starch (HES) 200/0.5 - Hemohes; HES 130/0.4 - Voluven; modified gelatin solution - Gelofusin) on the hemostatic system in 36 bone marrow donors (healthy individuals). The solutions were used in combination with crystalloid solutions during a procedure to compensate for intraoperative blood loss. Hemostatic changes were noted by the end of an operation in all groups; however, these were less pronounced when Voluven was administered. Thus, all colloid infusion solutions have varying effects on the hemostatic system, with a tendency toward both hypo- and hypercoagulation. According to TEG, HES 130/0.4 (Voluven) has a minimal effect on the hemostatic system.

[Pulmonary extravascular water in patients with acute respiratory failure].

The purpose of the investigation was to study pulmonary extravascular water levels and pulmonary vascular permeability (PVP) in the pathogenesis of acute respiratory failure (ARF)/acute respiratory distress syndrome (ARDS). Twenty-nine patients with ARF/ARDS and 10 healthy volunteers were examined. Central hemodynamics and oxygen transport were explored, by using a Swan-Ganz catheter. Intrathoracic volemic parameters were studied by the transpulmonary thermodilution technique. PVP was assessed by the pulmonary 67Ga-labelled transferrin leakage index. Plasma colloid osmotic pressure (COP) was measured on an osmometer. In most patients with ARF/ARDS, the pulmonary extravascular water index (PEVWI) was found to be higher (mean 16.9 +/- 1.5 ml/kg). At the same time its value was not greater than 10 ml/kg in 7 (24%) of 29 patients. There were no correlations between PEVWI and PaO2/FiO2 and between pulmonary extravascular water and AaDO2. The PVP index (PVPI) measured by transpulmonary thermodilution was 3.2 +/- 0.2, it being normal in 13 (45%) out of 29 patients. The pulmonary 67Ga-transferrin leakage index was higher in all the patients than in healthy individuals (23.2 +/- 2.9 x 10(-3) vs 5.7 +/- 9.9 x 10(-3)) and correlated with PaO2/FiO2 (r = 0.71; p = 0.01). In patients with ARF/ARDS, COP was lower (19.9 +/- 0.7 mm Hg). There were correlations between COP and PEVWI (r = -0.34; p = 0.01), COP and PVPI (r = -0.40; p = 0.044), COP and PaO2/FiO2 (r = 0.35; p = 0.02). PEVWI correlated with the COP-pulmonary wedge pressure gradient (r = -0.45; p = 0.0024). Hypoxemia correlated with intrapulmonary shunt (Qs/Qt). There was no relationship between Qs/Qt and PEVWI in the group as a whole. According to the ratio of Qs/Qt to PEVWI, the patients were divided into 2 groups. Group 1 comprised 11 patients with the ratio < or = 2; Group 2 included 18 patients with the ratio > or = 2, i.e. with an unproportional shunt enlargement as to the severity of pulmonary edema. A correlation between Qs/Qt and PEVWI was found in both groups: r = 0.82; p = 0.001 with the ratio < or = 2 and r = 0.48; p = 0.04 with the ratio > or = 2. Diverse causes of shunt formation were histologically detected. Thus, pulmonary edema was not identified in 24% of patients with ARF/ARDS. Arterial hypoxemia is associated with the increase in the shunt, but, in a portion of patients, the shunt was caused with atelectasis unassociated with pulmonary edema. Increased pulmonary permeability for transferrin is detectable in ARF/ARDS irrespective the severity of pulmonary edema. The pathogenetic features of lung lesions should be taken into account while choosing a treatment for ARF/ARDS.

[Artificial lung ventilation using separate intubation of two main bronchi in patients with acute respiratory failure].

Two-luminal endotracheal tubes are used for one-lung or selective ventilation. However, in some cases there is a need for not only to isolate the lung, but also to exclude the trachea from ventilation, for example, in injuries of its distal portions. The use of a two-luminal endobronchial tube under these conditions does not always permit isolation of an affected area since the tracheal cuff is frequently above the site of an injury. For the treatment of such complications and for selective ventilation, it is proposed to use separate intubation of two main bronchi under guidance of fibrobronchoscopy. For this, two single-luminal bronchial tubes having different inlet openings are simultaneously used; one tube is inserted translaryngeally, the other is placed through the tracheostomic opening. The tube having a cut in the bronchial cuff with an opening for ventilation of the right upper lobar bronchus is employed for the right main bronchus. There are examples how to use separate intubation of the main bronchi in patients with acute respiratory failure. This procedure may be the method of choice for treating tracheal distal injuries and, if required, for performing selective artificial ventilation. The effects of the latter are analyzed.

[The efficacy of artificial pulmonary ventilation in acute respiratory failure in patients with hemoblastoses]. / Effektivnost' iskusstvennoi ventiliatsii legkikh pri ostroi dykhatel'noi nedostatochnosti u bol'nykh gemoblastozami.

The authors review pulmonary effects achieved for 3 years in hemoblastosis patients subjected to artificial lung ventilation (ALV). Out of 44 patients treated the response to ALV was obtained in 7 (16%). Prior to ALV, two of them had granulocyte count under 0.5 x 10(-9)/1. ALV was discontinued in them after recovery of normal granulocyte count. Three patients (7%) survived 6 months and longer. Problems of differential diagnosis in different variants of acute respiratory failure in hemoblastosis patients and ALV potentialities are discussed.

[Use of meropenem in patients with neutropenia]. / Primenenie meropenema u bol'nykh s neitropeniei.

Meropenem was used in the treatment of 14 infectious complications in 11 patients including 8 with acute myeloid leukemia due to the cytostatic therapy, 1 with chronic myeloid leukemia, 1 with aplastic anemia and 1 with acute intermittent porphyria. At the moment of the meropenem use critical neutropenia (less than 500 granulocytes per 1 ml) in 11 cases (79 per cent) was stated. The drug was administered as intravenous infusions in a dose of 1 g every 8 hours for 4 to 41 days (the median of 11 days). 9 out of the 14 infectious complications were cured (the body temperature normalized and all the inflammation foci were eliminated), among them 6 out of 8 pyocyanic sepsis. Eradication of gram-negative bacteria was observed in 8 out of 10 cultures of the biological materials. No toxic complications or electrolytic disorders due to the drug use were recorded. The drug tolerance was good.

[Atypical electrolytic disorders in patients with diseases of the blood system]. / Netipicheskie élektrolitnye narusheniia u bol'nykh s zabolevaniiami sistemy krovi.

AIM: To describe and analyze rare iatrogenic disturbances of water-electrolyte and acid-base balances in systemic blood diseases. MATERIAL AND METHODS: Five cases of life-threatening electrolytic imbalance resultant from therapy in patients with hemoblastoses and a patient with acute intermittent porphyria (AIP). RESULTS: Hyperkalemia arose in 2 patients treated with cyclosporin A and trimethoprim-sulfamethoxasole. One patient developed severe arrhythmia because of hypomagnesemia following long-term therapy with aminoglycosides and amphotericin B. In one AIP patient hyponatremia ending in coma was attributed to the disease activity and infusion therapy. This was the cause of coma. In a patient with acute leukemia and pneumonia amphotericin B therapy entailed distal renal tubular acidosis and marked dyspnea which was primarily mistaken for acute respiratory failure. Pathogenetic, diagnostic and therapeutic aspects of the above water-electrolyte defects are provided. CONCLUSION: The appearance of water-electrolyte imbalance in hemoblastosis patients calls up a detailed analysis of the going-on therapy whether the imbalance is iatrogenic.

[Possibility of diagnosis and treatment of thrombotic complications in primary mediastinal B-cell lymphosarcoma]. / Vozmozhnosti diagnostiki i lecheniia tromboticheskikh oslozhnenii pri pervichnoi mediastenal'noi B-kletochnoi limfosarkome.

AIM: Efficacy of the treatment of primary mediastinal B-cell lymphosarcoma depends to a great extent on early diagnosis and treatment policy. In this study we evaluated possibilities of diagnosis and treatment of thrombotic complications of primary mediastinal B-cell lymphosarcoma (PMBL). MATERIAL AND METHODS: 61 patients were examined using roentgenography, computed tomography, chest ultrasound investigation,coagulogram, allelle specific polymerase chain reaction, ultrasound investigation of the jugular, subclavian, brachial veins, vena cava superior to detect mutation of genes II, V factors and methylentetrahydrofolatereductase. RESULTS: In 7 cases prechemotherapy examination detected thrombosis of the internal jugular and subclavian veins. In 4 of 7 cases there was a combined thrombosis of the left internal jugular and subclavian veins, in 3 cases one the vessels was affected with thrombosis. In 2 cases, in the course of polychemotherapy, there was recurrent thrombosis and development of pulmonary artery thromboembolism (PATE). In progression of the disease there was thrombosis of the left subclavian vein (1 case) and PATE (a case). Coagulologically, hypercoagulation syndrome signs were registered. 5 patients with PMBL complicated by thromboses showed gene mutations. CONCLUSION: In PMBL there is a tendency to formation of venous thrombosis and development of PATE. This is explained by tumor process and hereditary factors of thrombogenicity. Therefore, specific antitumor treatment should include anticoagulation therapy.