[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.

[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.

[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.

[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.

[First experience with the modified program NHL-BFM-90 application in adult patients with primary diffuse large B-cell gastric lymphosarcoma with unfavourable prognosis].

AIM: To assess efficacy of a modified program NHL-BFM-90 in adult patients with primary diffuse large B-cell gastric lymphosarcoms (PDLBGL) with unfavourable prognosis. MATERIAL AND METHODS: Modified courses of NHL-BFM-90 were conducted in 5 patients aged 27-67 years from January 2004 to September 2005. Four patients received chemotherapy of the first line, in one patient block therapy followed monotherapy with chlorambucil and a CHOEP course. All the patients were in a severe clinical condition and had several initial factors of unfavourable prognosis: size of the tumor more than 10 cm; stage IE and more advanced; B-symptoms; proliferative activity above 70%. The program NHL-BFM-90 was modified because of the patients' age. Chemotherapy was conducted according to the middle arm of the original program NHL-BFM-90, but methotrexate was introduced in a dose 1 g/m2 for 12 hours, while leukovorin was given 18 hours after the start of methotrexate injection. In two cases the blocks were enhanced with rituximab, 2 patients had doxorubicin in block A, in one case block C was enhanced with methotrexate. A total of 23 modified blocks NHL-BFM-90 were performed: one patient was given 6 blocks, two patients--5, one patient--4 blocks and one patient--3 blocks. RESULTS: Four patients after block 2 and one patient after block 3 of polychemotherapy NHL-BFM-90 achieved remission of the disease of 6 to 22 months duration which still continues. Infectious complications related to hematological toxicity arose more frequently at the latest courses of chemotherapy. CONCLUSION: Treatment according to the modified program NHL-BFM-90 in adult patients with PDLBGL and unfavourable prognosis is highly effective. For a mean follow-up of 10.2 months no recurrences occurred. The number of courses can be reduced to decrease accumulated hematological toxicity and in case of rapid achievement of remission.

[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.

[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.