In 185 patients with chronic hemoblastosis (chronic lymphocytic leukemia, chronic myeloid leukemia, idiopathic myelofibrosis, multiple myeloma) after autopsy, the pathology of the bronchopulmonary system was studied. It was found out that in addition to immunodeficiency, an important role in the occurrence of respiratory diseases in chronic lymphocytic leukemia, as well as in chronic myeloid leukemia and idiopathic myelofibrosis in the stage of blast crisis is played by specific leukemic infiltration of the lungs, bronchi, pleura and diaphragm; the presence of leukostasis in the vessels of medium and small caliber with violation of microcirculation; compression of the diaphragm by significantly increased spleen and liver; in some cases (especially in chronic lymphocytic leukemia) hyperplasia of the lymphoid follicles of the bronchial tree. In chronic myeloid leukemia and idiopathic myelofibrosis, hyperthrombocytosis with the development of the sludge syndrome in small vessels of the lungs is essential. Pulmonary localization of inflammatory processes in patients with multiple myeloma is facilitated by lymphoid and plasma cell infiltration of the lungs, paraproteinosis of the lungs, localization of myeloma nodes in the ribs, lung tissue and bronchi.

Hematogenous thrombophilia is often encountered in the clinical practice of doctors of all specialties. The article presents current data on the diagnostic of this pathology. In most cases pulmonary embolism is a clinical onset of hereditary hematogenous thrombophilia. According to the authors of this article, pulmonary embolism occurred in 60% of surveyed patients with hereditary thrombophilia hematogenous. Current antithrombotic therapy is quite effective in this category of patients. Three cases of personal experience of the authors when there was diagnosed a combined form of hematogenous thrombophilia with serious thrombotic complications in anamnesis are shown. Adequate secondary prevention of thrombosis contributed to further disease-free course of the disease.

Целью исследования явилось изучение морфо-функционального состояния бронхолегочной системы у больных множественной миеломой (ММ) на разных этапах опухолевой прогрессии. Комплексно были обследованы 103 пациента с ММ. При проведении морфологического исследования легких, бронхов, плевры 65 умерших от ММ установлено, что патоморфологическими проявлениями миеломатозного поражения бронхолегочной системы являются парапротеиноз легких (58 %), специфическая плазмоклеточная и лим- фоидная инфильтрация легких и бронхов (40 %), пневмосклероз (78,5 %), эмфизема легких (78,5 %), амилоидоз (13,8 %), миеломатозное поражение плевры (6,5 %), при развитии почечной недостаточности - нефрогенный отек легких (80 %), уремический пневмонит (28 %), кальциноз (25 %). У больных ММ на поздних этапах опухолевой прогрессии отмечается значительное снижение экскурсии диафрагмы при спокойном и форсированном дыхании вследствие ее миеломатозного поражения и остеодеструктивного процесса в грудной клетке. У пациентов с IIIА стадией ММ и при присоединении хронической почечной недостаточности вследствие развития остеодеструк- тивного процесса в плоских костях грудной клетки, специфического миеломатозного и уремического поражения бронхолегочной системы, нарушения экскурсии диафрагмы, при проведении спирографии, диагностировано нарушение вентиляционной функции легких по рестриктивному и смешанному типам. У 52 % больных ММ на поздних этапах опухолевой прогрессии диагностированы гипоксемия и легочная гипертензия. При проведении энодобронхиальной лазерной допплеровской флоуметрии установлено, что в процессе опухолевой прогрессии при ММ вследствие повышенной вязкости крови и анемического синдрома нарушаются показатели эндобронхиальной микрогемоциркуляции.

Based on the literature data and our own observations, the features of the lung damage in patients with drug dependence have been studied. It was found out that the pulmonary manifestations of drug addiction are secondary, depend on the type of drug used and ways of drug administration. In most cases, pneumonia develops, characterized by an aggressive course, often complicated by the formation of destructive cavities, suppuration and empyema of the pleura, respiratory failure. Pneumonia is often a manifestation of sepsis (in most cases, injecting drug users) and is accompanied by other syndromes: bacterial endocarditis, renal, hepatic insufficiency, and other manifestations of multiple organ pathology. Interstitial pneumonia can develop with the development of pneumofibrosis, bullous pulmonary degeneration, granulomatous inflammation, pulmonary edema. In a number of cases (more often with inhalational drug use) toxic alveolitis may develop. The defeat of the lungs can be irreversible and lead to death. Three clinical observations from the personal practice of the authors are presented.

The aim of this study was a retrospective analysis of the effectiveness of the domestic Factor VIII drug Octofactor for the prophylactic treatment of patients with hemophilia A living in the Amur Region. For the past 3 years, 10 hemophilia A patients over the age of 18 years have been receiving replacement therapy with this medication while other 10 hemophilia A patients of the same age have been receiving Factor VIII, a foreign drug. Each group included 6 patients with severe hemophilia A and 4 patients with moderate disease. This retrospective clinical study has been approved by the local Ethics Committee Amur State Medical Academy, Ministry of Healthcare of Russian Federation. All of the patients have signed voluntary informed consent for the use of their personal data in clinical studies and publications. Octofactor at a dose of 20–40 IU/kg body weight was administered every second or third day (2 to 3 times a week). Prevention has significantly reduced the need for hospitalization, improve performance. Patients with good adherence to treatment of spontaneous bleeding were not observed. Spontaneous bleeding of mild to moderate severity was diagnosed only in patients with low adherence to treatment, was stopped by administering the drug in therapeutic doses on an outpatient basis and did not require hospitalization. It was possible to achieve the desired indicators of clinical control – no more than 2 spontaneous hemarthrosis or other bleeding per year. Adverse reactions were not identified in patients treated with the drug Octofactor. With prophylactic treatment, it was possible to reduce the number of bleeding by 98%, both in patients using foreign commercial drugs of Factor VIII and in the treatment with Octofactor, compared with the period until 2005, when patients with hemophilia A received treatment on demand with cryoprecipitate and freshly frozen plasma. It is concluded that Octofactor preparation is not inferior to foreign Factor VIII concentrates in terms of efficiency and safety and can be effectively used for the prevention of bleeding in hemophilia A, including for individualized therapy