[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.

[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.

[Transfusion-associated lung injury (TRALI): obvious and incomprehensible].

Acute transfusion-associated lung injury (TRALI) is an acute lung injury associated with and develops within 6 hours after the transfusion of components and blood preparations. Today there are no uniform views on the pathogenesis of TRALI. The discussion of immune and non-immune mechanisms is relevant. The key link of the former is that the presence of anti-leukocytic antibodies in a donor or a recipient and their interaction during transfusion with the leukocytes of the recipient or the donor, respectively; that of the latter link is the accumulation of biologically active substances in the transfusion media during storage and their passive administration to the recipient during transfusion. In both cases, the total link is drastic increased pulmonary capillary permeability. The clinical presentation of TRALI is nonspecific and generally similar to that of the adult respiratory distress syndrome and lung injuries of another genesis. It is necessary to make its differential diagnosis with allergic reactions, the transfusion of bacterially contaminated media and mainly with circulatory overload. Specific treatments for transfusion-associated lung injury are unavailable. Diferent variants of respiratory therapy are effective. Prevention of TRALI is mainly based on its immune mechanism. The leading direction of its prevention is to select donors.

[Enhancement of coagulation caused by transfusion of artificial plasma-replacement solutions].

AIM: To investigate hemostasis disorders caused by massive blood transfusions of artificial plasma replacing solutions (PRS). MATERIAL AND METHODS: Two groups of patients were examined: 7 healthy volunteers without blood loss (group 1) and 11 healthy donors of bone marrow with intraoperative blood loss 1-2 l (group 2). Five patients of group 1 received transfusion of 12 ml/kg hydroxyethyl starch (HES) 130/0.4, two patients of group 1 received transfusion of modified gelatine solution (gelofusin). All of them received infusions (1-1.5 l) of crystalloid PRS (1-2 l) and infusion of one of colloid PRS (6-HES, 5--gelofusin). Estimated hemodilution in group 1 was 1.17 +/- 0.01 times, in group 2 it varied from 1.3 to 2.7 times (mean 1.78 +/- 0.4 times). Hemostasis was studied by clot growth rate (for groups 1 and 2), endogenic thrombin potential and parameters of thromboelastography (for group 2) in plasm samples obtained before, 2.24 and 48 hours after infusion of colloid PRS. RESULTS: For both groups spatial clot growth rate 2 hours after hemodilution was high. Then it fell and reached baseline level 48 hours after PRS infusion. Endogenic thrombin potential and thromboelastography data (for group 2) changed by the same pattern. A hypercoagulation effect of gelofusin on parameters of thromboelastography and clot growth rate was higher than of HES 130/04. CONCLUSION: Moderate hemodilution with PRS in vivo causes hypercoagulation which persisted longer than volemic effect of PRS.

[Comparative evaluation of different tracheal accesses for artificial ventilation of the lungs in patients with depressed hematopoiesis]. / Sravnitel'naia otsenka razlichnykh dostupov k trakhee dlia provedeniia IVL u bol'nykh s depressiiami krovetvoreniia.

The purpose of this study was to analyze the complications and errors in tracheal intubation and tracheostomy, development of algorithm of steps for provision of endotracheal access in patients with hematological diseases. Provision of endotracheal access in 115 patients with blood system diseases is analyzed retrospectively. A total of 113 orotracheal, 50 nasotracheal intubations, and 48 tracheostomies were carried out. In 6 patients fibrobronchoscope was used for intubation because of pronounced hemorrhagic syndrome, inflammatory changes, and soft tissue edema of the face and neck; in 2 patients tracheostoma was inserted under local anesthesia. In 12 patients nasotracheal intubation was complicated by nasal bleeding. The most frequent and serious complication in patients with a tube in the trachea was obturation of the tube with sputum (9.7% of orotracheal intubations, 30% of nasotracheal intubations, and 15% of tracheostomas). Extubation/decannulation was carried out in 20 patients. One patient developed cicatricial stenosis of the trachea after tracheostoma. Hemorrhagic tracheobronchitis was endoscopically diagnosed most often (in 55 cases). Autopsy showed hemorrhages in the mucosa in 68.2%, in soft tissues adjacent to tracheostoma in 41.4%, and suppurative inflammation in 15.2% cases. Hence, hemorrhagic tracheobronchitis, hemorrhages in the mucosa, soft tissues of the trachea and bronchi are the most frequent clinical and morphological complications in patients with depressed hemopoiesis. Indications for nasotracheal intubation in this patient population should be limited. Early tracheostomy is recommended. Algorithm of steps ensuring the patency of airways in patients subjected to forced ventilation of the lungs is offered.

[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.

[Multiple-organ pathology in septic shock in patients with hemoblastoses]. / Poliorgannaia patologiia pri septicheskom shoke u bol'nykh s gemoblastozami.

A prospective study of 20 patients with hemoblastosis and septic shock (SS) was carried out by invasive monitoring of the central hemodynamics and oxygen transport, evaluation of biochemical and coagulological parameters, and assessment of the severity of clinical condition by the APACHE II and SOFA scores. Septic shock was effectively treated in 12 patients, 5 of them were discharged from the department (group 1) and 7 died in intensive care wards from various complications (group 2). Eight patients died during the first 2 days from SS resistant to therapy (group 3). Group 2 patients were in need of a longer inotropic support than group 1 patients (5.8 +/- 1.6 vs. 2.7 +/- 0.8 days, p < 0.01). The deficit of bases was more expressed in groups 2 and 3 in comparison with group 1 (-11.3 +/- 3 and -2.7 +/- 9.1 mmol/liter vs. 1.4 +/- 4.4 mmol/liter) and left ventricular stroke index (LVSI) and oxygen delivery were lower. LVSI and base deficit were in linear correlation (rho = 0.4, p < 0.05). XIIa-dependent fibrinolysis was suppressed in all patients, which was more pronounced in group 3 in comparison with groups 1 and 2 (135 +/- 47.4 vs. 103 +/- 27 and 88.3 +/- 42.3). According to SOFA score, the severity of cardiovascular disorders during day 1 of SS was the same in all groups, while starting from day 2 it decreased in patients who survived. Acute respiratory failure was lower in group 1 only on day 1 according to SOFA. More pronounced (according to SOFA) hepatorenal failure was observed in group 2 in comparison with other patients. Organ involvement in hemoblastosis was detected at autopsy in 8 out of 13 cases. Hence, the need in prolonged cardiovascular support of SS patients is associated with development of polyorgan involvement. Fibrinolysis suppression is a frequent early manifestation of hemostasis disorders. Specific neoplastic organ involvement was observed in 61.5% patients with hemoblastosis who died from SS and its complications.