[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.

[Investigation of ß-endorphin concentrations in patients with hemophilia].

UNLABELLED: AIM. To study changes in the plasma concentration of beta-endorphin (beta-E) in patients with hemophilia A and B (in the presence of bleeding and in the absence of hemorrhagic syndrome) and in whole blood and plasma donors before and after donation and to investigate the factors associated with (beta-E) concentration changes. SUBJECTS AND METHODS: The prospective study of beta-E concentration changes (and related factors) enrolled 38 persons: 12 patients with hemophilia after acute blood loss, 11 patients with hemophilia without hemorrhagic syndrome, and 15 whole blood and plasma donors. beta-E concentrations were measured by enzyme immunoassay. RESULTS: In blood loss, the patients with hemophilia were found to have elevated serum beta-E concentration: 9.6 pg/ml (95% confidence interval (CI), 6.1 to 13.0 pg/ml) versus 5.2 pg/ml (95% CI, 1.4 to 8.9 pg/ml). After donation, the concentration of 3-E in the group of donors was higher than before donation: 7.3 pg/ml (95% CI, 4.9 to 9.7 pg/ml) versus 4.7 pg/ml (95% CI, 3.2 to 6.3 pg/ml). In the group of patients with hemophilia, the elevation of 3-E concentrations is steady-state (lasted at least 10 days); at this time, the beta-E value variability (estimated by mean square deviation) increased as compared with that in remission: 7.7 pg/ml (95% CI, 5.5 to 13.1 pg/ml) versus 2.4 pg/ml (95% CI, 1.7 to 4.4 pg/ml). The above differences are statistically significant (p = 0.05). CONCLUSION: In blood loss, there is an increase in plasma beta-E concentrations in the patients with hemophilia and donors. The increase in beta-E concentrations and the variability of its values were greater in the patients with hemophilia and blood loss than in the donors. The beta-E concentration elevation accompanying hemorrhage is characterized by steadiness in the patients with hemophilia.

[Spleen removal in hematological patients with splenomegaly].

By large and giant volume of the spleen the mainstay of the surgery remains the open splenectomy. Splenomegaly stipulates certain difficulties and dangers of the procedure, mainly because the lack of the free space in the abdominal cavity. The maximally wide access, preliminary preparation and ligation of the splenic artery in situ and thorough separation of the spleen and pancreatic tail provide the safety and success of the operation. 374 splenectomies on the reason of the splenomegaly were analyzed. The standardization of the technique allowed the 2.5 times reduction of the postoperative complication rate (from 26.9 to 10.8%); the 1.8 times reduction of the intraoperative complication rate and the 17% reduction of the intraoperative blood loss. The postoperative pancreatitis was registered 4 times rare; the intraabdominal bleeding - 2 times rare and the frequency of the subphrenic abscess was 1.5 times lesser.

[Splenectomy in programmed therapy of aplastic anemia].

AIM: To define efficacy of splenectomy (SE) in current programmed therapy of aplastic anemia (AA). MATERIALS AND METHODS: SE efficacy was analysed in 2 stages: a retrospective study of efficacy of surgery as monotherapy (1986-1996) (74 AA patients) and of SE in programs of immunosuppressive therapy (IST) (1991-2002). Program treatment of AA patients was conducted on the base of IST algorithm developed in Hematological Research Center after many year investigations. RESULTS: SE as monotherapy improved AA course in 73.3% patients with non-severe AA (NAA) and 18.2% patients with severe AA (SAA). Three and five year survival in NAA postsplenectomy patients was 80%. One-year survivors after surgery were likely to survive long. Overall survival of SAA after SE was significantly less (p < 0.0001): 3-year survival - 6%. SE efficacy in programs including antilymphocytic globulin (ALG) and cyclosporin A (CsA) was studied in 69 AA patients. A 85.5% response was registered to program treatment including ALG, CsA and SE, being 81% in SAA and 1% in NAA patients. Efficacy of SE in combination with CsA at the first stage NAA treatment (a 30% positive response) was much inferior to ALG+CsA (68% response). At stage two treatment SE improved treatment results in most of SAA patients. Long-term survival in SAA patients after program treatment with SE is 60%. CONCLUSION: SE in the program of combined therapy in adult AA patients including CsA is an alternative to ALG in NAA patients. In SAA, SE can be included in the program at the first stage in ALG intolerance or in the absence of the drug, at the second stage--to overcome resistance to conducted therapy.

[Preoperative embolization of the splenic artery in patients with blood system diseases]. / Predoperatsionnaia émbolizatsiia selezenochnoi arterii u bol'nykh s zabolevaniiami sistemy krovi.

AIM: To develop and introduce X-ray endovascular preocclusion of the splenic artery in general surgical patients with different forms of cytopenias as preparation for further surgical treatment. MATERIALS AND METHODS: Data on 12 splenic arterial occlusions in patients with different diseases of the blood system. RESULTS: Despite the severity of the underlying process, the use of X-ray endovascular preocclusion of the splenic artery in patients with different hematological diseases made it possible to make the so-called bloodless splenectomy on short notice, then a required cavitary operation or in the immediate postembolization period if there were emergency indications or some time later if the patient's status allowed. CONCLUSION: When patients with blood system diseases attended by thrombocytopenia, anemia, leukopenia are indicated to have surgical treatment of some contaminant diseases, it is necessary to perform X-ray endovascular splenic arterial occlusion at the first stage, then make a major intervention when blood parameters have improved.

[Lung biopsy in the diagnosis of causes of lung involvement in hemoblastosis]. / Biopsiia legkogo v diagnostike prichin porazheniia legkikh u bol'nykh gemoblastozami.

AIM: To ascertain the role of lung biopsy in diagnosis of lung lesions in hemoblastosis (HB) patients. MATERIAL AND METHODS: The results of diagnostic biopsies of the lungs obtained from 22 HB patients are presented. Ten patients had no respiratory insufficiency (RI), twelve patients had RI. The biopsy was transbronchial in 1 case, thoracoscopic in 10 and open in 11 cases. RESULTS: In RI-free patients lung biopsy was informative in all the cases. The biopsy provided information which allowed therapy modification resulting in improvement of the patient condition. In RI patients biopsy was informative in 8 of 12 patients. Nonspecific changes in the lungs were identified histologically in 2 of 12 patients. In 2 RI patients lung biopsy confirmed the diagnosis made after examination of the bronchoalveolar lavage. Modification of therapy after the biopsy was conducted in 58.3% HB patients with RI. Improvement was seen in 2 of them. 10 of 12 patients with RI died within 1-2 weeks after biopsy. CONCLUSION: Lung biopsy in HB patients should be obtained only after examination with noninvasive methods and before development of RI as prognosis after lung biopsy in the presence of RI is unfavourable. The histological material should be examined for all expected pathogens.