Macroscopically, focal, focal-confluent, and polysegmental pneumonia, on the background of pronounced pulmonary edema, looked like compaction areas ranging in size from 1 to 5 cm in diameter, from grayish-red to dark-red. These areas protruded above the surface of the incision of the lungs. Histologically, in the alveoli, exudates with a predominance of fibrin and a small number of neutrophils were found. There were signs of circulatory disorders on the periphery of the alveoli filled with exudate. A number of patients were diagnosed with resorption of fibrinopurulent exudate and growth in these areas of granulation and connective tissue in the form of Masson’s bodies (carnification, organization). Changes in the bronchi were local in the area of ​​chronic inflammation. Peribronchial changes prevailed: peribronchial tissue thickening,deformation and constriction of the bronchi with unevenly expanded gaps. In areas of carnification, clusters of alveolar macrophages and desquamated type 2 alveolocytes, the formation of granulation tissue inside the alveoli and respiratory bronchioles, and focal pulmonary fibrosis were detected in the lumen of the alveoli. The organization of exudate in the alveoli was detected against the background of marked infiltration of the interalveolar septa with lymphocytes. Clutches of lymphocytes were formed around small vessels. Often, interlobular, intersegmental, peribronchial fibrosis and obliteration of the terminal and respiratory bronchioles were noted. Microscopic examination of lymphoid infiltration of interalveolar septa, signs of “organizing pneumonia” and obliterating bronchiolitis were detected by microscopic examination of the majority of patients who died of CLL with severe pneumonia.In blood vessels of various calibers, leukostasis was noted with infiltration of the vessel walls with lymphocytes. In the centers of carnification and pneumosclerosis, cavities having a rounded or convoluted shape were often detected. These cavities were islands of preserved alveoli. In the areas of granulation tissue, the formation of a large number of capillaries was noted. In a number of pneumosclerosis foci, in a number of patients, vascular reduction and the appearance of arteriovenous anastomoses were detected. Morphological changes in the lungs (lymphoid infiltration of the interalveolar septa, impaired microhemocirculation and tissue trophism, “organized pneumonia”, bronchiolitis obliterans) contributed to the severe and prolonged course of pneumonia in these patients.In the centers of carnification and pneumosclerosis, cavities having a rounded or convoluted shape were often detected. These cavities were islands of preserved alveoli. In the areas of granulation tissue, the formation of a large number of capillaries was noted. In a number of pneumosclerosis foci, in a number of patients, vascular reduction and the appearance of arteriovenous anastomoses were detected. Morphological changes in the lungs (lymphoid infiltration of the interalveolar septa, impaired microhemocirculation and tissue trophism, “organized pneumonia”, bronchiolitis obliterans) contributed to the severe and prolonged course of pneumonia in these patients.In the centers of carnification and pneumosclerosis, cavities having a rounded or convoluted shape were often detected. These cavities were islands of preserved alveoli. In the areas of granulation tissue, the formation of a large number of capillaries was noted. In a number of pneumosclerosis foci, in a number of patients, vascular reduction and the appearance of arteriovenous anastomoses were detected. Morphological changes in the lungs (lymphoid infiltration of the interalveolar septa, impaired microhemocirculation and tissue trophism, “organized pneumonia”, bronchiolitis obliterans) contributed to the severe and prolonged course of pneumonia in these patients.In a number of pneumosclerosis foci, in a number of patients, vascular reduction and the appearance of arteriovenous anastomoses were detected. Morphological changes in the lungs (lymphoid infiltration of the interalveolar septa, impaired microhemocirculation and tissue trophism, “organized pneumonia”, bronchiolitis obliterans) contributed to the severe and prolonged course of pneumonia in these patients.In a number of pneumosclerosis foci, in a number of patients, vascular reduction and the appearance of arteriovenous anastomoses were detected. Morphological changes in the lungs (lymphoid infiltration of the interalveolar septa, impaired microhemocirculation and tissue trophism, “organized pneumonia”, bronchiolitis obliterans) contributed to the severe and prolonged course of pneumonia in these patients.

In all CLL patients who died when pneumonia was attached, there was an increase in the lymph nodes in the chest cavity, 14 of them had sarcomal transformation of these lymph nodes. Enlarged, sarcomotransformed, dense lymph nodes with Richter syndrome squeezed the lung tissue and bronchi, which contributed to impaired lung ventilation function, bronchial drainage function and the emergence of the inflammatory process.

As an example, here are two extracts from archival case histories.

Extract from the case history No 14267. Patient G., born in 1936. Diagnosis: chronic lymphocytic leukemia, tumor form, stage C according to the classification of J. Binet. The diagnosis of CLL was made in 2002, confirmed by histological examination of the lymph node and immunophenotyping of peripheral blood lymphocytes (CD5 +, CD19 +, CD20 +, CD22 +, CD23 +). Concomitant diseases: ischemic heart disease, circulatory failure IIA.

Upon admission to the hospital (March 12, 2006), there is an increase in the lymph nodes of all peripheral groups up to 5–6 cm in diameter with a densely elastic consistency, soldered between each other, form “packages”, with surrounding tissues are not soldered, without painful. Packings of enlarged abdominal lymph nodes are palpable through the anterior abdominal wall. The lower edge of the liver is palpated 8 cm below the costal arch. The spleen is palpable 13 cm below the left costal margin. In the lungs, vesicular breathing and wheezing are not heard. Heart sounds are muffled, the rhythm is right. Blood pressure 150/90 mm. Hg Art.HR – 88 beats per minute. A blood test (from 13.03.2006): hemoglobin – 68 g / l, red blood cells – 2.7 × 10 12 / l, leukocytes – 166.7 × 10 9 / l, platelets – 90 × 10 9 / l, lymphocytes – 98%, segmented – 2%, ESR – 10 mm / h. CCFG: lungs without focal and infiltrative shadows, the roots of the lungs are enlarged. ERTGS – an increase in mediastinal lymph nodes. Immunogram: CD 3 – 29.0%, CD 4 – 19.1%, CD 8 – 19.0%, CD 4 / CD 8 – 1.0, CD 20 – 63.1%, CD 22 – 30.2 %, immunoglobulins: A – 1.1 g / l, M – 1.0 g / l, G – 8.0 g / l. The patient underwent a course of therapy with fludararabine (Fludar tablet tablets, at a dose of 30 mg / m 2 , 1 – 5 days) in combination with cyclophosphate (at a dose of 200 mg / m 2 , 1 – 5 days), red blood cell transfusions, etc. Positive dynamics was observed: the lymph nodes of all peripheral groups decreased to 2–2.5 cm in diameter. The size of the liver and spleen has decreased. The lower edge of the liver protrudes 5 cm from the edge of the costal arch, the lower edge of the spleen is palpable 4 cm below the left costal edge. A blood test (from March 25, 2006): hemoglobin – 104 g / l, erythrocytes – 3.3 × 10 12 / l, leukocytes – 27.9 × 10 9 / l, platelets – 100 × 10 9 / l, lymphocytes – 96%, segmented – 4%, ESR – 18 mm / h. The patient notes improvement in well-being, preparing for discharge. On March 28, 2006, the temperature suddenly rose to 39 С. Chills. Tachycardia up to 120 beats per minute. Severe weakness, indisposition, shortness of breath at rest, feeling of lack of air. A decrease in blood pressure to 100 and 50 mm Hg is noted. During auscultation in the lower parts of the right lung moist rales are heard. An x-ray examination of the lungs was performed (March 29, 2006). Conclusion: focal-confluent pneumonia in the lower lobe of the right lung. In the clinical analysis of blood from March 29, 2006, the number of leukocytes decreased to 2.5 × 10 9 / l. KHS and blood gas composition: pH – 7,323; pCO 2 – 48.6 mm. Hg Art., pO 2 – 56.6 mm. Hg Art., O 2 SAT – 88%, O 2 CONT – 12.7 vol /%, AaDO 2 – 44.9 mm. Hg Art. In the sputum and bronchial washings, the pathogen was not detected in the pathogenic titer. Empirical therapy with broad-spectrum antibiotics was performed. Initially, the combination of ciprofloxacin (0.4 × 2 p / day, IV drip) and medocef (2.0 × 2 p / day, IV drip) was prescribed, followed by the combination of sulperazone (4.0 × 2 p / day, iv drip) and amikacin (1.5 g. 1p. per day). Immunomodulatory (immunoglobulins), antimycotic (mycosyst, mycoflucan) and detoxification therapy were prescribed. Blood pressure values ​​normalized on the fifth day, dyspnea stopped on the 10th day of treatment, the temperature returned to normal on the 18th day, wheezing stopped listening on the 16th day. Weakness, malaise persisted. 04/29/2006, a repeated X-ray of the lungs was taken.On control radiographs in the frontal and lateral projections, a decrease in pneumonic infiltration in the lower part of the right lung was determined (S 4-5-10). Conclusion: right-sided foci-confluent pneumonia in the resolution stage. For 4 weeks, pneumonia was not completely resolved, took a protracted course. The treatment was continued – a combination of fortum (2g. × 2 p / day, intravenous droplet) and ciprofloxacin in the same dose (given the good effect of prescribing this antibiotic). Immunomodulatory and detoxification therapy was continued. On radiographs from 13.05.2006, infiltration is not determined. As a complication, the development of focal pneumofibrosis is noted. Full normalization of well-being (the absence of all extrapulmonary manifestations of pneumonia) was also noted at the beginning of May 2006.In this case, classical nosocomial pneumonia took place in a patient with CLL that developed after a course of polychemotherapy, which has a heavy and protracted course.

Extract from the case history No 4239. Patient K., born in 1955. Diagnosis: Chronic lymphocytic leukemia, progressive form, stage C according to the J. Binet classification. CLL was diagnosed in 2004. Clinical blood test from 2004. – hemoglobin – 135g / l, erythrocytes – 3.9 × 10 12 / l, leukocytes – 35 × 10 9 / l, lymphocytes 73%, choke-core – 2%, segmented core – 20%, monocytes – 5%, platelets – 180 × 10 9 / l. Immunophenotype of peripheral blood lymphocytes – CD5 +, CD19 +, CD20 +, CD22 +, CD23 +. Expressed expression of CD38 was not observed at that time. Peripheral lymph nodes of all groups in 2004 did not exceed 1.5 – 2 cm. Liver, spleen were not enlarged. At that time, the stage A CLL was classified according to the Binet classification. Within two years, there was no significant progression of hemoblastosis, cytostatic therapy was not prescribed, and patients were monitored dynamically. Deterioration since September 2006: peripheral lymph nodes, liver, spleen began to increase; leukocytosis increased progressively; anemia and thrombocytopenia increased. Blood count: hemoglobin – 89g / l, erythrocytes – 2.78 × 10 12 / l, leukocytes – 134.16 × 10 9 / l, lymphocytes 86%, prolymphocytes – 8%, segment-nuclear – 6%, platelets – 70 × 10 9 / l. The patient was prescribed mono-therapy with fludarabine, subsequently the FCR protocol. But a persistent therapeutic effect was not observed. In June 2007 Clinical and X-ray studies diagnosed destructive pneumonia in the S 6 segment. left. Pulmonary tuberculosis was excluded (sputum and bronchial lavages at KUM, CT of the lungs were repeatedly examined, consultation with a phthisiologist). From the bronchial washings, S. pneumoniae and neisseria were sown. In the treatment of pneumonia, a combination of broad-spectrum antibiotics was used to identify the causative agent; after the causative agent was detected, antibiotic therapy was corrected taking into account its sensitivity. Immunomodulatory, antimycotic, anti-inflammatory therapy was prescribed. Fully clinical and radiological manifestations of pneumonia resolved in August 2007. October 18, 2007 He was taken to the therapeutic department of the CRH (at the place of residence) by an ambulance in a critical condition. There was an increase in body temperature to 39 – 40 ̊С, a decrease in blood pressure, severe shortness of breath. Clinical blood test:red blood cells – 2.4 × 10 12 / l, hemoglobin – 67 g / l, leukocytes – 274 × 10 9 / l, lymphocytes – 87%, prolymphocytes – 10%, segmented cells – 3%, platelets – 50 × 10 9 / l, ESR – 61 mm / h . During the X-ray examination, pneumonia was again diagnosed in the S 6 segment on the left. Despite the conduct of adequate antibacterial, anti-inflammatory, immunomodulating, detoxification therapy, 20.10.2007. a lethal outcome was established with the phenomena of bacterial toxic shock. Histological examination of tissue S 6 the left lung revealed lymphoid infiltration of the pulmonary interstitium; in the alveoli exudate was found with a significant predominance of fibrin, a small number of neutrophils, accumulations of bacteria. In this case, the focus of inflammation was localized in the area of ​​lymphocytic infiltration of the lung, which, along with pronounced immunodeficiency, caused severe and recurrent pneumonia.

Based on a study of the clinical and morphological features of the course of pneumonia in CLL patients, the following conclusion can be drawn : 1. Pneumonia is the most common infectious complication of CLL (103 patients out of 228 examined, which is 45%). As CLL progresses, the incidence of pneumonia increases. Patients with CLL have a high incidence of nosocomial pneumonia (36%).
 
 
 For patients with CLL, the prevalence of prolonged (53.4%), severe (60%) and atypical (27.6%) course of pneumonia is characteristic. The causes of atypical, severe, and protracted pneumonia in CLL are: a) pronounced secondary immunodeficiency, primarily neutrophil deficiency and hypo-immunoglobulinemia, b) courses of chemotherapy and glucocorticoid therapy, which contribute to the development of an ammunition deficit, aggravating the development of the ammunition and corticosteroids, which contribute to the development of an inadequate case of chemotherapy and glucocorticoid therapy, which contribute to the inadequacy of cortical diseases, which contribute to the development of an obstructive, severe and prolonged course of pneumonia in CLL; lung tissue and bronchi, d) significant microcirculation in the lungs and bronchi, e) pulmonary ventilation disturbance and perfusion, e) elderly rast of most CLL patients, g) presence of concomitant diseases (COPD, IHD, diabetes, etc.), pulmonary ventilation function and bronchial drainage function. Gram-negative flora occupies an important place among pneumonia pathogens in patients with CLL – 56% of the total number of hospital pneumonia and 26.3% of cases of community-acquired pneumonia. In accordance with the occurrence of pneumonia in the hospital or outside the hospital, the suspected causative agent, the clinical and pathogenetic situation and the presence of underlying diseases, an empirical antibiotic therapy of pneumonia has been compiled in patients with CLL
 identifying the pathogen and determining its sensitivity to antibiotics, as well as for those situations when it is impossible to establish the etiological diagnosis of pneumonia.

Regional ventilation and lung perfusion were studied using reopulmonography. MEP of the right lung: in the upper zone – 8.3, in the middle zone – 7.8; in the lower zone – 7.2 ohm / min; MOPP of the left lung: in the upper zone – 8.1, in the middle zone – 7.7; in the lower zone – 7.6 ohm / min. MOVr sum = 46.7 ohm / min. The ratio of MOV of the upper zones / MOV of the lower zones = 1.1, which indicates a significant redistribution of ventilation from the lower zones of the lungs to the upper ones. MPCr of the right lung: in the upper zone – 10, in the middle zone 8.2, in the lower zone 6.8; MPCr of the left lung: in the upper zone – 9, in the middle zone 8.6, in the lower zone 6.1 Om / min; MPKr sum 48.7 ohm / min. Reprinted pulmonary blood flow from the lower zones to the upper ones due to the increase in vascular resistance in the lower and middle zones and its decrease in the upper zones. VPO were distributed as follows: right lung – upper zone 0.83, middle zone 0.95, lower zone 1.0; left lung – upper zone 0.9, middle zone – 0.9, lower zone 1.2. The total index of the HPE of the right lung is 0.9, the left lung is 1, both lungs are 0.95. The data of ultrasound IWC: SrDLA – 24 mm. Hg Art., KDO PZh – 135 ml, CSR PZh – 51 ml, UI PZh – 72 ml / m 2 , SI PZH – 4.3 l / min / m 2 , EF RV – 54%, E TK – 0.44 m / s, A TK 0.70 m / s, E / A – 0.63. Diagnosed the expansion of all cavities of the heart. Ultrasonic examination of the diaphragm: TD – 5 mm, EDS – 8 mm, EDf – 13 mm. A decrease in the excursion of the diaphragm with quiet and forced breathing was diagnosed, which is due to its compression by significantly increased liver and spleen.

After analyzing the data of spirography, peak flow measurements, pneumotachography, and zonal rheography of the lungs, these studies of endobronchial LDF, pulmonary and intracardiac hemodinamics in patients with CLL, we can conclude:

1. In the process of tumor progression in CLL, indices of endobronchial microhemocirculation decrease. Important causes of endobronchial microcirculation disorders in CLL are high leukocytosis in the peripheral blood, contributing to the formation of leucostasis; in patients with anemic syndrome, a decrease in the number of erythrocytes.

2. Disruption of microhemocirculation in patients with CLL contributes to disruption of tissue trophism, development of tissue hypoxia, local metabolic disorder, resulting in the onset of a severe and prolonged inflammatory process in the lungs and bronchi and an increase in pressure in the small circle of the circulation. When conducting a fibrobronchoscopic study in 60% of patients with a progressive course of CLL, the latent course of chronic non-obstructive bronchitis was diagnosed (40% of the total number of CLL patients).

3. As the tumor process develops in CLL, violations of general and regional ventilation and lung perfusion are progressed, which is characterized by a decrease in these indicators in each zone separately and in general in both lungs. There is a redistribution of ventilation and blood flow from the lower and middle zones to the upper zones of both lungs. These changes are due to the presence of infectious complications and leukemic bronchopulmonary manifestations of CLL, a violation of the diaphragm excursion.

4. In patients with CLL in the later stages of the tumor progression, bronchial resistance increases due to severe and severe inflammatory processes, leukemic infiltration of the lungs and bronchi, impaired endobronchial micro-hemocirculation, compression of the bronchial tree by increased mediastinal lymph nodes.

5. As the CLL progresses, there are impaired systolic and diastolic functions of the right and left ventricles.

6. Violation of the contractile ability of the diaphragm in patients with CLL is promoted by its leukemic lesion and compression by the enlarged liver and spleen.

7. In 37% of patients with CLL in the late stages of tumor progression without a concomitant broncho-obstructive process, hypoxemia and pulmonary hypertension are diagnosed. A disturbance in the expansion of the diaphragm, a severe course of infectious complications and leukemic lesions of the bronchopulmonary system, rheological disorders in the ICC vessels, and myocardial dystrophy contribute to an increase in pressure in the LA system in these patients.

Among the 228 patients examined for CLL, chronic obstructive pulmonary disease (COPD) was diagnosed in 50 patients (21.9%). There was a significant predominance of men compared with women (44 and 6 people, respectively). The average age was 59.5 ± 3.2 years. All have a history of long-term smoking. The diagnosis of COPD in all patients was made before the first clinical and laboratory signs of CLL appeared. The duration of COPD is from 5 to 20 years. In group I, 7 patients with COPD were diagnosed, in group II, 30, in group III, 13 people.

The main clinical manifestations of COPD were dyspnea, cough, sputum production (predominantly mucopurulent or purulent), sweating, weakness, fatigue, increased body temperature , etc. . It should be noted that the last four symptoms may be a manifestation of CLL, with its progressive course (patients of groups II and III). Therefore, in all such situations, we tried to clarify whether these symptoms are a manifestation of COPD, CLL, or the associated symptoms of both diseases. Symptoms of tumor intoxication in CLL appear in the later stages of tumor progression, when there are other signs of progression of the tumor process – an increase in the size of lymph nodes, a progression of leukocytosis, an increase in anemia and thrombocytopenia, lack of effect from cytostatic therapy, etc. Choking, coughing in patients with Richter syndrome, due to compression of the lung tissue and bronchi, dense lymph nodes were rarely seen. therefore, their presence in all cases was explained by COPD. Massive leukemic infiltration of the lungs, which can be distinguished radiographically, the clinical manifestation of which may be shortness of breath, is very rarely diagnosed with CLL . Dyspnea in patients with CLL group III may be a clinical manifestation of anemic syndrome. In such patients, they tried to stop anemia as quickly as possible, if shortness of breath persisted, regarded it as a symptom of COPD. When associating COPD with CLL, auscultational picture and spirography are of great diagnostic value. In CLL, even in the case of a significant increase in bronchopulmonary lymph nodes, there were no violations of VFL detected by the method of spirography .

In patients with benign CLL, there are no symptoms of tumor intoxication and anemia, therefore, patients from group I have shortness of breath, sweating, weakness, fatigue, increased body temperature (in the absence of other comorbidities) explained by COPD.

Diagnostic bronchoscopy was performed in 40 patients with COPD. In 8 people, a double-sided diffuse endorchitis of I degree was detected, in 15 – II degree and in 17 – III degree.

Mean respiratory function recorded by spirography in patients with COPD in combination with CLL (VCD – 54.5 ± 3.8% D, FEV 1 – 50.9 ± 3.4% D, ICT – 51.7% ± 3, 5D, POS vyd – 34.8 ± 2.5% D, MOS 25 – 32.6 ± 3.0% D, MOS 50 – 31.9 ± 2.0% D, MOS 75 – 39.1 ± 3, 2% D) did not have significant differences with similar indicators in patients with COPD without concomitant hemoblastosis (3rd control group: VC 54.9 ± 3.2% D, FEV 1 – 51.5 ± 2.5% D , IVT – 52.8 ± 2.8% D, POS vyd – 35.3 ± 2.0% D, MOS 25 – 32.9 ± 2.0% D, MOS 50 – 32.7 ± 2.9% D, MOS 75 – 40.2 ± 4.0% D).

In accordance with the spirometric classification of COPD, depending on the indicators of FEV 1 and FEV 1 / FVC [262, 322], 21 (42%) patients of the II stage (moderately severe), 17 (34%) patients were diagnosed in the CLL patients group Stage III (severe) and 12 (24%) patients with Stage IV (extremely severe). In the 3rd control group, 11 patients were diagnosed II, 9 patients III and 5 patients with IV stages of COPD, which in percentage terms corresponded to patients of the main group (44, 36 and 20%, respectively).

A study of the magnitude of bronchial resistance by the method of pneumotachography in patients with HO BL occurring on the background of CLL showed a significant increase in R aw compared with similar indicators in the control group (healthy) and a slight increase compared with the data of patients with COPD without an associated lymphoproliferative disease. Once contrast performance values Bron hialnogo resistance at Paci ENTOV primary and third control groups can be explained by the fact that in some patients of Group III at baseline, these figures are slightly higher due to specific bronchial pulmonary manifestations limfoley goat and latent course of chronic Cesky obstructive bronchi that.

On average, in the morning, the PSV indices, with peak flowmetry, were 47% D and in the evening 52% D. Changes in the HRP within a day did not significantly exceed 5%. There were no significant differences in the PSV indices and its daily fluctuations in patients of the main and 3rd control group.

In order to study microhemocirculation in the proximal bronchial tree, 10 patients with COPD associated with CLL (2 patients from I, 6 from II, and 2 from III groups) underwent endobronchial LDF on a laser analyzer of capillary blood flow LAAC-02. Disorders of microcirculatory blood flow were diagnosed in all patients. PM has been significantly reduced. The σ values ​​were lower than the control values. Kv was significantly higher than control. Correlations were found between the intensity of inflammation in the bronchi and a decrease in PM (r = –0.57; P <0.05), between a decrease in FEV 1 and PM (r = 0.46; P <0.05). However, it should be borne in mind that in patients with CLL outside of the attachment of AML, there is an initial decrease in PM.

During the amplitude-frequency analysis of the Dopplerograms, it was found that the amplitudes of oscillations in the E-band, in patients with COPD were lower than the control values ​​(P <0.01). This can be explained by a decrease in endothelial oscillations in patients with COPD, without concomitant hemoble astrosis [238], since in patients with CLL, outside the administration of AML, there are no changes in endothelial oscillations .

The amplitude of oscillations in the H-band, associated with the sympathetic effects on smooth muscle cells of arterioles and arteriolar regions of arterio-veno-vulvar anastomoses was increased compared with the control. An increase in the amplitude of oscillations in this frequency range indicates arteriolar vasodilation, which can be explained by the presence of a broncho-obstructive process, since the majority of COPD patients have severe and moderate degrees who do not have hemoblastosis and increased neurogenic fluctuations. Dilation of arteriol can be an adaptive response of the microcirculatory bed, since it contributes to the optimal blood flow to the capillaries in patients with leukostasis.

The amplitudes of oscillations in the M and D bands, indicating the activity of local x pacemakers inside the smooth muscle cells of the precapillary sphincters and venous tone, did not differ from the control.

The amplitude of oscillations in the C-band, brought into the microcirculatory bed from the arteries, was reduced compared with the control. A decrease in the amplitude of oscillations in the C-band indicates a decrease in the flow of arterial blood into the microvasculature. This may be due to microcirculation disorders in the bronchi in patients with CLL, a large role, in the development of which belongs to the presence of leucostasis. In many patients, peripheral blood leukocytosis of more than 100 × 10 9 / l was observed . It must be borne in mind that in patients with CLL, outside the attachment of AML, during the tumor progression, a decrease in the C-band oscillation amplitudes is diagnosed. At the same time, in patients with COPD, without concomitant hemoblastosis, no changes in cardiac fluctuations were diagnosed .

4 weeks after initiation of COPD therapy, these patients were given endobronchial LDF again . The PM indicator remained significantly reduced. The values ​​of σ and Kv did not undergo significant changes. The amplitude of endothelial oscillations increased slightly and did not have significant differences with the control (in contrast to the initial indicator). The amplitude of oscillations in the neurogenic range remained elevated. The amplitude of cardiac oscillations remained almost unchanged and was reduced. The amplitudes of oscillations in the myogenic and respiratory ranges did not significantly change. Etc. oanalizirovav WMS data but to conclude that significant changes in the microcirculatory blood flow in the mucous membrane of the proximal bronchi in patients with COPD associated with CLL remain, even if the bronchoscopic picture improves. This may be due, primarily, to the presence of lymphocytic leukemia, in which initially there are places of significant impairment of micro hemocirculation. In many patients with CLL, without a concomitant broncho-obstructive process, PM, Ac are significantly reduced . Disorders of microhemocirculation contribute to the recurrence of severe and prolonged inflammatory processes in the lungs and bronchi, resulting in an increase in pressure in the pulmonary circulation.

The gas composition of blood was studied in patients with COPD flowing on the background of CLL and COPD without an associated lymphoproliferative disease. In the main group, there was a significant increase in pCO 2 , a decrease in pO 2 and capillary blood pH, as compared with the control. Indicators of blood gas composition in patients of the main and third control groups did not have significant differences.

Clinical and radiological manifestations of pulmonary emphysema were diagnosed in 40 patients with COPD. The clinical manifestations of emphysema in these patients were the following symptoms: the chest is cylindrical barrel-shaped, the lower borders of the lungs are lowered by one or two ribs, the smoothness of the supraclavicular depressions, expansion and bulging of the intercostal spaces, reduction of the respiratory excursion of the lungs, boxed sound over the entire lung surface , with auscultation, weakened breathing, reduction of cardiac dullness, visible pulse tion in the epigastric region. All of these patients had shortness of breath, aggravated by exertion, and increased fatigue. In patients with pulmonary emphysema, a significant loss of body weight was observed. However, in patients with CLL, with a rapidly progressive course of the disease and in the terminal stage, cachexia may be a manifestation of hemoblastosis.

The radiographic signs of emphysema were as follows: barrel-shaped or bell-shaped chest with horizontally extending posterior rib segments and extended intercostal spaces; the vertical size of the chest is enlarged, the sternum is deflected anteriorly, the retrosternal space “gapes” (more than 3-5 cm); an increase in the area and transparency of the pulmonary fields; discharge, impoverishment of the pulmonary pattern; in the inspiratory position low aperture; costal diaphragmatic sinuses flattened, deployed; the excursion of the domes of the diaphragm is sharply reduced. The most reliable radiological signs of pulmonary emphysema are detected by X-ray computed tomography .

During spirography, there was a decrease in VC (48 ± 5.2% D), an increase in the functional residual capacity of the lungs, and a residual volume. Mild emphysema (residual volume of 35–45%) was diagnosed in 21 patients, severe (45–50%) in 11 patients and severe (over 55%) in 8 people.

It should be noted that the number of patients diagnosed with some clinical and radiological signs of emphysema (98 people, including 40 patients with emphysema caused by COPD) is greater than the number of patients with emphysema in COPD (Chapter 3). This is due to the elderly patients with CLL, in this situation there is a so-called. senile emphysema. Unlike true emphysema, in patients with COPD, the air capacity of the lungs in these patients did not decrease, pulmonary hypertension and right ventricular hypertrophy did not develop.

Immunological studies were carried out in 40 patients with COPD associated with CLL during the period of exacerbation of the disease and at 14-15 days after the start of treatment. A comparative analysis of the immunological parameters of the main and control groups was carried out. In patients with CLL, there is a significant decrease in the phagocytic activity of neutrophils, a decrease in the content of serum immunoglobulins of classes A, M and G, and immunosuppression in T-lymphocyte content. These changes are explained by the B-cell tumor nature of the disease – B-CLL. For the same reason, for 14–15 days, after the start of treatment, there were no changes in the indices of cellular and humoral immunity . Immunodeficiency dictates the need for immunomodulating therapy in the treatment of COPD in patients with CLL.

The exacerbations of COPD in patients with a progressive course of CLL often coincided with courses of polychemotherapy (CP, COP, CHOP, ATS, FC, FCR, FCM), which contributes to the progression of immunodeficiency. In patients with CLL with association with COPD treated with chlorambucil and cyclophosphamide exacerbations of the broncho obstruction was not observed.

During exacerbation of COPD in patients with progressive CLL, the clinical symptoms of which disease determine the severity of the condition at the moment were assessed. In most cases, these were symptoms of COPD. In such situations, first of all, therapy was directed to the treatment of the broncho-obstructive process and only upon achieving remission, COPD was prescribed cytostatic therapy. The appointment of aggressive chemotherapy in the period of exacerbation of COPD, in all cases led to an even greater burden of bronchopulmonary disease, and even to the development of pneumonia in patients with CLL.

The course of COPD on the background of CLL is characterized by a significant inhibition of immunity, a more severe course and a slow dynamics of clinical syndromes. All modern methods of treatment of COPD have been used to treat these patients . The dynamics of clinical manifestations of COPD in patients with CLL in the stage of exacerbation of the obstructive process in the lungs was compared with the dynamics of these indicators in patients with COPD without CLL (3rd control group) . Due to the characteristics of CLL, which is characterized by leukocytosis and absolute lymphocytosis in blood tests, indicators of the number of leukocytes and neutrophils were not taken into account when assessing the severity and dynamics of clinical manifestations of COPD during the exacerbation.

Of the 228 patients with CLL, 40 patients were diagnosed with chronic pulmonary heart (CID) according to clinical, radiographic, electrocardiographic, and echocardiographic studies. In all 40 people, the cause of the development of HPS was a prolonged course of chronic obstructive pulmonary disease (COPD). There were no diseases with a primary lesion of the pulmonary vessels and with thoracodiaphragmatic disorders in this group. In all patients, the duration of diagnosis of COPD ranged from 5 to 20 years. In all 40 patients, according to clinical and radiological data, pulmonary emphysema occurred, in 25 patients according to x-ray data, diffuse pneumosclerosis.

Clinical manifestations of HPS were detected in 40 patients . Compensated pulmonary heart was diagnosed in 28 and decompensated in 12 people. In patients with compensated HLS, the symptom complex of chronic respiratory failure prevailed. Dyspnea, fatigue during exercise, cyanosis in these patients for a long time were only manifestations of respiratory failure. An important role in the timely diagnosis of HPS in these patients was played by given target physical, laboratory and instrumental examinations. Physical examination revealed the following symptoms: an enhanced diffuse cardiac impulse in the pericardial region; tachycardia; accent II tone over the pulmonary artery her; amplified I tone over the tricuspid valve compared to the apex of the heart; amplified II tone above the xiphoid process, at the point of attachment of the IV – V ribs to the sternum to the right compared to the base of the heart; epigastric pulsation; the displacement of the right border of the relative dullness of the heart; a push of the right ventricle in the IV – V intercostal space, on the left at the edge of the sternum; presystolic or protodiastolic gallop in the zone of auscultation of the tricuspid valve or right ventricle.

Clinical manifestations of decompensated pulmonary heart were an enlarged liver (it must be borne in mind that an enlarged liver in this situation may be a clinical manifestation of CLL, therefore, when diagnosing decompensated HPS in these patients, hepatomegaly did not have an important diagnostic value), edema of the lower extremities, ascites , swelling of the cervical veins, pulsation of the liver, positive symptom of Plesch, systolic murmur at the base of the sternum, nocturia, oliguria, complications from the central nervous system and Stems Thus , the formation of decompensated pulmonary heart was characterized by adherence of right ventricular heart failure to respiratory failure.

Radiographic signs of CID in patients with CLL were bulging of the cone of the pulmonary artery and expansion of the right heart, which were detected in 32 patients.

All patients had more than two “direct” signs of pulmonary heart on an ECG: 1) Rv 1 ≥ 7 mm; 2) R / Sv 1 ≥1 mm; 3) Rv 1 + Sv 5 ≥ 10.5 mm; 4) the time of internal deviation in the lead V 1 ≥ 0.03-0.05 s; 5) QR complex in lead V 1 ; 6) incomplete blockade of the right bundle of His bundle with Rv 1 ≥ 10 mm; 7) complete blockade of the right bundle of His bundle with Rv 1 ≥ 15 mm; 8) right ventricular loading in V 1 – V 2 management (inversion of T wave).

During echocardiography and ICDH, pulmonary hypertension was diagnosed in 40 patients with COPD complicated by CPH (during ECG and ICDD, there was an increase in pressure in the LA system, hypertrophy and / or dilation of the right ventricle were detected), and 20 patients with CLL were not diagnosed. who had a history of COPD and were not abused by smoking (37% of the total number of CLL patients without COPD). In 10 patients with CLL with concomitant COPD (diagnosed according to clinical and instrumental data), pulmonary hypertension was not detected (10%).

Patients with CLL associated with COPD, and without a broncho-obstructive process, who, according to echocardiography and IDGC, were diagnosed with LH, are divided into four subgroups: subgroup A – 10 patients with CLL with COPD without LH signs; subgroup B – 28 patients with compensated HPS; subgroup C – 12 patients with decompensated HPS; subgroup D – 20 patients with CLL without COPD, but with the presence of LH signs. For comparison, an ultrasound study of the heart was carried out in patients with COPD and HPS without concomitant lymphoproliferative disease (additional control subgroups): 10 people with COPD without PH ( control subgroup A KONT ), 25 people with COPD and signs of HLS in the stage compensation (control subgroup B KONT ), 25 people with COPD and signs of HPS in the decompensation stage (control subgroup C KONT ). Patients of the control subgroups by age and sex matched the patients with CLL.

TMPS of the pancreas in patients of subgroup A differed from the same indicator in the control (P <0.01). In the stage of compensated pulmonary heart, the thickness of the myocardium of the right ventricle reached maximum values ​​(compared to control, subgroups A and C – P <0.001). In case of decompensation of the CHPS, the TMPS PZH exceeded the control indicator with a reliability of <0.001, but was significantly less than in the compensation stage (P <0.001). In subgroup D, the myocardial thickness of the pancreas was significantly different from the control indices (P <0.001) and subgroup B (P <0.001). TMPS prostate patients subgroup A Comte , it was smaller than in patients of the subgroup A (P <0.01). Increase TMPS PZH in patients with COPD occurring on the background of CLL and not complicated by pulmonary hypertension, it can be explained by tumor intoxication, cardiotoxic effects of cytostatics, anemia, lymphoid myocardial infiltration. The TMPS indicators of the pancreas of subgroups B and B KONT , C and C KONT did not have significant differences .

There was an increase in the indices of the CRV of the pancreas in patients with CLL in combination with COPD and HLS in subgroups B and C (P <0.001). In patients with compensated and decompensated CPH, an increase in the RER RV is associated primarily with prolonged pulmonary hypertension, due to the presence of a pathologic process in the lungs. No significant differences in the CRD of the main pancreas and the corresponding control subgroups were found.

In the study of the functional ability of right heart diseases in patients of subgroup A, there was a decrease in E TK (P <0.05) and a significant increase in A TK (P <0.001), respectively, the ratio E / A TK (P <0.001) decreased. Other hemodynamic parameters were not significantly different from the control data. Compared with subgroup A KONT , the A value of TK was significantly increased and the ratio E / A decreased (P <0.001), which is explained by an increase in heart rate at the late stages of tumor progression in CLL, due to anemia and intoxication .

In subgroup B, there was an increase in BWW of the pancreas (P <0.001), CSR pancreas (P <0.001), a decrease in PV of the pancreas (P <0.01). Compared with the control, E TK was significantly reduced (P <0.05), A TK increased (P <0.001). Revealed a decrease in E / A TC (P <0.001). Compared with subgroup B KONT , there were significant differences in the indices A TC and E / A (P <0.001) .

In patients with decompensated CPHC on the background of CLL (subgroup C), the diffuse reflectance and CSR of the pancreas were significantly increased (P <0.001); EF significantly decreased the prostate (P <0.001), IAs pancreas (P <0.01), SR pancreas (P <0.05). Patients in subgroup C showed a significant decrease in E TK (P <0.01), an increase in A TK (P <0.001) and a decrease in the E / A ratio (P <0.001). Significant differences of A TK and E / A compared with subgroup C KONT were noted. Due to significant dilatation of the pancreas, the SR pancreas was reduced .

In patients with HPS, the main role in the violation of hemodynamics of the ICC belongs to prolonged pulmonary hypertension, leading to morphological changes in the ICC vessels and in the right ventricular myocardium.

In subgroup D, an increase in the CRD of the pancreas (P <0.05), an increase in KDO (P <0.05) and CSR (P <0.05) of the pancreas were diagnosed. An increase in the SI of the pancreas was noted (P <0.001) due to an increase in the heart rate; an increase in A TK (P <0.05), a decrease in E TK (P <0.05) and, accordingly, a decrease in the ratio E / A (P <0.001). In this group, a violation of pulmonary hemodynamics, systolic and diastolic functions of the pancreas is a consequence of hemoblastosis ( myocardial dystrophy, lymphoid infiltration of the myocardium, etc.), and not bronchopulmonary pathology. Ane mia, intoxication, lymphoid infiltration of the myocardium in patients with CLL, in the later stages of tumor progression, promote different vitiyu not adequate circulation . Myocardial dystrophy caused many violations of pulmonary hemodynamics in subgroup D, including an increase in pressure in the aircraft system.

In the study of the contractile ability of the left ludochka in patients with COPD associated with CLL of subgroup A, an increase in A MK was diagnosed (0.5 ± 0.03 m / s; P <0.01), and a decrease in the E / A LV ratio (1 , 2 ± 0.05 m / s; P <0.001), not typical for patients of subgroup A KONT . This is due to the initial increase in A MK in patients with CLL, due to an increase in heart rate. The remaining indices of the ultrasound study of the left ventricle corresponded to those of healthy individuals and subgroup A KONT . Patients diagnosed in the sub-group of reliable, as compared to control increasing A MK (0.53 ± 0.03 m / s; P <0.001) and, accordingly, a decrease in the E / A ratio (1.0 ± 0.05; P <0.001). No significant difference was found in other indicators of the LV contractile function from those of the control and subgroup B KONT . In the study of the systolic function of the left ventricle in patients of subgroup C, a decrease in the contractile ability of the LV was recorded . Compared with the control, the differential diffuse reflectance and LV CSR were increased (143 ± 5.8. P <0.05 and 60.5 ± 3.0, P <0.01 ml; respectively), EF decreased (61 ± 2, 7%; P <0.01). There was a decrease compared with the control of LV LV (35.6 ± 1.6; P <0.05), LV SI (2.8 ± 0.05 l / ml / m 2 ; P <0.05). Significant differences of these indicators with those in subgroup C CONT is not revealed. In subgroup C, a significant increase was diagnosed compared with the control and subgroup C KONT , A MK (0.55 ± 0.02 m / s; P, respectively, <0.001 and <0.01), which is explained by an increase in heart rate in these patients, and respectively, a decrease in E / A (P <0.001). Thus, LV diastolic dysfunction in patients with COPD associated with CLL has been observed even in patients with normal pulmonary artery pressure. But dilatation of the left ventricle and

The development of its contractile ability develops only into the stage of decompensated HPS. Patients of subgroup D have diastolic and systolic LV dysfunctions (increase in BWW – 140 ± 5.0 ml, P <0.05; CSR – 55 ± 5.0 ml, P <0.05; And MC – 0.58 ± 0.03, P <0.001, a decrease in E MK – 0.53 ± 0.02; E / A – 0.93 ± 0.5, P <0.001) and an increase in SI LV – 3.6 ± 0.25 l / ml / m 2 , P <0.05 (due to increased heart rate).

During ultrasound examination of the diaphragm in patients of subgroup A, the thickness of the diaphragm did not change significantly. The position, shape, echogenicity of the diaphragm did not differ from those in the control group. There was a significant decrease in the excursion of the diaphragmatic muscle with calm and forced breathing. Significant differences in the indices of TD, EDS and EDF in subgroups A and A KONT were not found. However, in subgroup A KONT , in contrast to subgroup A, the EMF did not significantly differ from the same in control. A more pronounced violation of EDS in CLL can be explained by an enlarged liver and spleen in patients with a tumor form of hemoblastosis included in this group, which contributed to the violation of the excursion of the diaphragm .

In patients with subgroup C, an increase in the thickness of the diaphragm was registered (P <0.001). The dome of the diaphragm was flattened, the echo structure became non-uniform. The mobility of the diaphragmatic muscle decreased not only with forced but also with quiet breathing. Significant differences in the indices of TD, EDS and EDF in subgroups B and B KONT were not found .

In patients with subgroup C, the dome of the diaphragm, during the ultrasound study, was fuzzy, slightly wavy, the echo structure was diffusely uneven, with foci of inclusions. The thickness of the diaphragm muscle was 5.5 ± 0.3 mm (P> 0.05). A decrease in the thickness of the diaphragm, as compared with subgroup B (P <0.001), is associated with dystrophic changes in this muscle. In this subgroup, the mobility of the diaphragm was significantly reduced. Her excursion decreased with calm and forced breathing. It should be noted that TD, EDS, and EDF differed significantly from those of subgroup B, which indicates a progressive diaphragm dysfunction. Significant differences in these indicators in subgroups C and C KONT were not found.

The results of the study allowed us to trace the development of changes in the functional state of the diaphragm in patients with CLL and COPD at different stages of the formation of the CID. An increase in the thickness of the diaphragm in patients with compensated HPS, a decrease in its thickness during decompensation of the HPS, and a decrease in the mobility of the diaphragm during calm and forced respiration indicate a progressive impairment of the functional state of the diaphragm as the COPD and HPS develop. Among patients with CLL, with the presence of concomitant COPD, the greatest changes in the functional capacity of the diaphragm were observed in patients with decompensated COP.

In subgroup D, the thickness of the diaphragm did not change, but its excursion during calm and forced breathing was significantly reduced . Violation of the excursion of the diaphragm in these patients is explained by its compression by significantly enlarged liver and spleen and leukemic damage ( lymphoid infiltration, leukostasis in vessels). It must be borne in mind that in subgroup D included a large number of patients with a significant increase in the liver and spleen.

Thus, it can be concluded that in patients with COPD without PH, the functional state of the diaphragm depends on impaired bronchial patency. In compensated drugs, the functional capacity of the diaphragm is impaired due to severe bronchial obstruction, arterial hypoxemia, pulmonary hypertension, and systolic and diastolic function of the pancreas. In patients with decompensated CPH, a significant decrease in the contractile ability of the pancreas joins the above. In patients with CLL, without concomitant COPD, with the presence of LH, the leading role in the disruption of the functional state of the diaphragm belongs to its compression significantly increased by the liver and spleen, and specific leukemic lesion.

Treatment of HPS in the stages of compensation and decompensation was carried out in accordance with modern guidelines .

SrDLA was measured prior to the initiation of appropriate therapy and after its termination. It was possible to achieve a decrease in SrDLA in patients with subgroups B (22.0 ± 2.6); C (27.7 ± 4.0); D (17.6 ± 1.1) mm. Hg Art. However, when conducting a statistical study, this decrease was not significant (P> 0.05).

After treatment in patients of subgroup A (COPD without LH), a significant decrease in the CSR of the pancreas compared to the baseline was observed – to 43.6 ± 2.5 ml (P <0.05), an increase in pancreas to 0.57 ± 0, 03 m / s (Р <0.05) and Е / А ratios – up to 1.03 ± 0.03 (Р <0.01).

In subgroup B (compensated for CPV), after treatment, there was a significant decrease compared to baseline in BWW of the pancreas (123.6 ± 2.0 ml; P <0.05), CSR pancreas (56.3 ± 2.2 ml; P < 0.05), an increase in the E / A ratio (1.18 ± 0.08; P <0.01).

In patients with subgroup C (decompensated pulmonary heart), after therapy, there was a decrease compared with baseline BWW of the pancreas and CSR pancreas,respectively, to 130.0 ± 4.1 ml (P <0.05) and 70.4 ± 4.1 ml (P <0.05). An increase in EF of the pancreas to 49.9 ± 2.9% (P <0.05), UI PZH to 39.2 ± 1.6 ml / m 2 (P <0.05), SI PZH to 3.1 ± 0.1 l / min / m 2 (P <0.01). On the part of the diastolic function of the pancreas, a significant increase in the pancreas E to 0.56 ± 0.04 m / s (P <0.05) was established; the E / A ratio to 0.9 ± 0.05 (P <0.01) However, these indicators after treatment were not normalized.

After treatment in patients of subgroup D (CLL with LH, but without concomitant COPD), an increase in the E / A ratio was observed to 1.34 ± 0.05 (P <0.05). The remaining hemodynamic parameters of the ICC before treatment did not significantly differ from the norm and remained practically unchanged after treatment.

The death was ascertained in 6 patients of subgroup A, 14 patients of subgroup B, 11 patients of subgroup C and 18 patients of subgroup D. According to the results of autopsy material, the morphology of the right ventricle myocardium, segmental bronchi, small circulation vessels, liver and diaphragm was studied in patients with CLL For comparison, data from autopsy material of the same organs of 30 practically healthy people who died from life-incompatible injuries (main control subgroup; control) and patients with COPD and HLS without an associated lympho-proliferative disease (additional control subgroups) were used: 10 people with COPD without signs CPH (control subgroup a to ) 23 persons with COPD symptoms and compensate CPH in step (subgroup B control to ), 30 people with COPD and signs of HPS in the decompensation stage (control subgroup C to ). Patients of the control subgroups by age and sex matched the patients with CLL.

A morphological study of segmental bronchi in patients with CLL deaths with COPD events identified four forms of chronic obstructive bronchitis: 1) catarrhal chronic bronchitis (CBC) was detected in 6 cases (20%) – in group A in 4 cases, in subgroup B in 2 cases; 2) catarrhal sclerosing chronic bronchitis (CACS) was detected in 12 cases (38%) – in subgroup A in 2 cases, in subgroup B in 6 cases, in subgroup C in 4 cases; 3) sclerosing chronic bronchitis (CHB) was detected in 10 cases (32%) – in subgroup B in 6 cases and in subgroup C in 4 cases; 4) granulating chronic bronchitis (HCB) – diagnosed in 3 cases (10%) in subgroup C. Summing up the morphological and morphometric studies of segmental bronchi in patients with COPD associated with CLL and COPD without concomitant hemoblastosis, it can be concluded that there are no significant morphological differences between these two cohorts of patients. An exception is the presence in patients with CLL with progressive, tumor and splenic forms of lymphoid infiltration disease. radios of the bronchi and the presence of clusters of lymphocytes in the vessels. In addition, in patients with CLL, due to the pathogenesis of the disease, the number of neutrophilic granulocytes in the cellular bronchial infiltrate is reduced. These changes can largely explain the protracted dynamics of COPD in patients with CLL. Lymphoid infiltration impairs the drainage function of the bronchi. Leukemia stasis and blood clots in the vessels of the bronchial wall lead to impaired microcirculation and blood supply to the wall of the bronchi. Disorders of microhemocirculation contribute to a severe, prolonged and recurrent course of COPD. A decrease in the number of neutrophils in the cellular infiltrate leads to a decrease in local immunity and contributes to a more protracted dynamics of the main COPD syndromes.

Indicators of the wall thickness of the pancreas, the width of the pancreas and the perimeter of the TSC in subgroups A and D were within normal limits. In subgroups B and C, their progressive increase was observed with a significant difference from control (P <0.001). In subgroup B, these changes are associated with concentric hypertrophy of the pancreatic wall and in subgroup C with dystrophic processes, which result in thinning of the wall and dilation of the pancreatic cavity with the development of relative tricuspid insufficiency. The LA circumference progressively increased from subgroup A to subgroups B and C, reaching a maximum in patients with decompensated CPH. The indicator of the circumference of the aircraft in the subgroup D with confidence <0.05 was different from the control. A significant increase in pancreatic mass was diagnosed in subgroups A, B, C, and D relative to the control (P <0.001). Ventricular index (LM) in all subgroups was significantly different from control. The absolute mass of the pancreas was significantly different from the control in all subgroups of the study. In subgroups A, B and D, microscopic examination of the pancreatic myocardium revealed fibromuscular hyperplasia and hypertrophic-hyperplastic processes, especially expressed in the stage of compensated CPHD. Atrophic and sclerotic processes prevailed in the stage of decompensation of the HPS. Significant differences in the above indicators among the patients of the respective main and additional control subgroups (COPD and HPS with CLL and COPD and HPS without concomitant hemoblastosis) were not found. but. At the same time, during the histological study of the myocardium of the pancreas in patients with CLL, additional control subgroups not characteristic of the patients were revealed : moderately severe lymphoid infiltration of the myocardium and accumulations of lymphocytes in the lumen of the coronary vessels. Lymphoid infiltration in the myocardium in CLL was significantly less pronounced than in the lungs, and was never detected in a macroscopic study .

In the study of the diaphragm thickness of patients of four subgroups, it was established that with an increase in the duration of the course of COPD and CID, the thickness increases , reaching maximum values ​​in the stage of compensated pulmonary heart. In the stage of decontamination of the CID, the thickness of the diaphragm decreased as compared with subgroups A, B and control. In patients with CLL with the presence of PH, but without a broncho-obstructive process in the lungs, the thickness of the diaphragm is slightly less than in the control group. Microscopic This study of the diaphragm revealed changes in myocytes and stroma. In all subgroups, mean myocytes prevailed. The greatest number of them was observed in subgroup A, in subgroups B and C there was a decrease in this indicator. A change in the number of large myocytes had a reverse tendency compared to the average muscle fibers, a greater number of them were diagnosed in patients with decompensated HPS. The same tendency was observed in the study of small muscle fibers. Chronic hypoxia, persistent energy hunger due to hyperfunction of the diaphragm as a result of COPD and HLS contributed to dystrophic and necrobiotic changes in the striped muscle fibers of the diaphragm. The cross-sectional hair contours with a long COPD course (subgroups B and C) looked less rounded,in places with retractions and bulges. In the stage of decompensated HPS, areas with necrosis of myocytes appeared. The number of stroma in subgroups A (12.2 ± 0.35%) and B (13.5 ± 0.4%) exceeded the control indicators (P <0.001), however, they did not differ significantly between them (P> 0.05 ). The largest number of stromal elements was observed in subgroup C – 28.4%, which significantly exceeded the control indicators of other study groups (P <0.001). In subgroup C in the stroma of the diaphragm there were areas of lipomatosis. Significant differences between morphometric data of the main and control subgroups (patients with COPD and PH without sopahowever, they did not differ significantly (P> 0.05). The largest number of stromal elements was observed in subgroup C – 28.4%, which significantly exceeded the control indicators of other study groups (P <0.001). In subgroup C in the stroma of the diaphragm there were areas of lipomatosis. Significant differences between morphometric data of the main and control subgroups (patients with COPD and PH without sopahowever, they did not differ significantly (P> 0.05). The largest number of stromal elements was observed in subgroup C – 28.4%, which significantly exceeded the control indicators of other study groups (P <0.001). In subgroup C in the stroma of the diaphragm there were areas of lipomatosis. Significant differences between morphometric data of the main and control subgroups (patients with COPD and PH without sopa lymphoproliferative disease) was not detected. Morphofunctional to artin reflects the compensatory-adaptive processes that occur in the diaphragm due to bronchopulmonary pathology and the hypoxia and hyperfunction caused by it. These factors lead to the development of hypertrophy, and then dystrophy of the striated myocytes of the diaphragm. With a long course of COPD and HPS, dystrophic changes reach a maximum, leading to fatty degeneration and necrosis of muscle fibers. In patients with CLL, in contrast to patients with CAS without lymphoproliferative disease, lymphoid infiltration of the diaphragm and lymphocytic stasis in the vessels of the diaphragm is noted . These changes, as well as compression of the diaphragm by the enlarged liver and splenic This contributes to impaired function of the diaphragm and the progression of the identified morphological changes . Morphological changes in the diaphragm in patients with subgroup D (CLL patients with PH, without COPD).

The morphological and morphometric studies of small circulation vessels (ISC) in CLL patients in combination with COPD at different stages of the formation of HLS were performed . The degree and frequency of identified changes increases with the progression of CPH. In patients with COPD and HLS, CLL in the arteries and arterioles of the ICC had changes of I – V degrees, in the veins – I – III degrees. Changes in the vessels of the ICC are characteristic of both the main patients (COPD on the background of CLL) and additional control subgroups (COPD without concomitant lymphoproliferative disease), at similar stages of the formation of the CPH. The morphological features of the vessels of the LA system in CLL patients were the following changes, which are not characteristic of patients with additional controls groups: filling gaps from vessels of different caliber with lymphocytes with the development of leukostasis, in some cases completely blocking the gaps of small vessels and causing a significant impairment of microcirculation ; in some patients, infiltration of the pulmonary vascular walls with tumor cells and multiple perivascular lymphoid cell lesions were noted.

In subgroup D, changes in the vascular ICC were less pronounced. In 13 cases, changes in arteries were noted and arterioles corresponding to the I degree (72.2%) – hypertrophy of the middle envelope, in 5 cases (27.8%) there were no changes. Changes in the veins were either absent or were not pronounced (I degree). We explain the less pronounced changes in the ICC vessels as compared with other subgroups by the shorter duration of PH in patients with CLL without COPD. Patients with I degree changes are mostly patients with significant liver enlargement and spleen. ki The duration of the disease is shorter than the duration of COPD in patients of the other subgroups. In patients without pathological changes in the vessels of the ICC, there was no significant increase in the liver and spleen. Consequently, an increase in pressure in the LA system in them is associated with a severe and prolonged course of inflammatory and specific leukoses in the lungs, an increase in lymph nodes in the chest cavity, and so on. That is, the duration of PH in this situation is even shorter and serious morphological changes detected in patients from other subgroups did not have time to occur in the vessels of the ICC.

Leukostasis in vessels in CLL patients in vivo is very difficult to identify, since there are no clinical and radiological manifestations of this complication, it is noted that leukostasis develops less frequently in patients with CLL than with acute leukemia and chronic myeloid leukemia. At the same time, impaired rheological indices of blood and pulmonary circulation is an important link in the pathogenesis of CLL. A decrease in pulmonary circulation is found in 97% of patients with CLL. The risk of developing leukostasis in the vessels of the lungs appears with an increase in the number of blood leukocytes above 50 × 109 / l, and with an increase of more than 200 × 109 / l, leukostasis almost always develops.

Pneumonia in CLL deserves special attention, because they are the dominant complication (up to 75%), playing a major role in the outcome of the disease. In a half of patients with CLL, a history of frequent pneumonia can be detected even before the onset of the disease. On the fact that pneumonia is the most common cause of mortality in patients with CLL, many authors point out. The occurrence of pneumonia is directly dependent on the presence of concomitant or previous pulmonary infectious process, i.e. leukemic infiltration of the lungs and bronchi. The pathogenesis of pneumonia may be different. Lymphoid infiltration of the bronchi can lead to their infection, the development of pneumonia and the death of a patient with CLL. The development of pneumonia in CLL can contribute to microcirculation disorders due to high leukocytosis.

Due to the development of lymphatic leukocytosis in B-CLL, these patients have varying degrees of severity of neutropenia. Belongs to pneumonia, occurring on the background of neutropenia, among the most severe, which often have a poor prognosis. They are characterized by complications: abscess formation, prolonged course, severe dysbiosis, sepsis. When the number of granulocytes in peripheral blood is <0.5 × 109 / l, the risk of developing infectious complications is higher than that of patients with a number of granulocytes> 1.0 × 109 / l, the risk of infectious complications is especially high with a level of granulocytes <0.1 × 109 / l [50]. With neutrophil count> 0.5 × 109 / l, a positive response to antibiotic treatment is observed in 90% of patients, and mortality is 7%, while with neutrophil count <0.5 × 109 / l – 60 and 20% respectively [50, 309, 355]. According to the WHO classification, there are 4 degrees of neutropenia. Especially dangerous is grade IV neutropenia (neutrophils less than 500 in 1 µl of blood), but patients with neutrophil count less than 100 in 1 µl are also isolated. The latter subgroup is the most threatened from the point of view of the development of fast-flowing gram-negative infection.

Of considerable interest is the work on the clinical and morphological features of pneumonia that occur in oncohematological patients against the background of myelodepression and neutropenia. The authors showed that for a microscopic picture of pneumonia on the background of myelodepression, there is a characteristic lack of manifestations of an inflammatory cell reaction in the lung tissue due to a sharp decrease in neutrophilic granulocytes in the circulating blood. In the lung tissue, exudation prevails, extending along the interalveolar passages without forming an inflammatory wall, limited only by anatomical structures (interlobar pleura). In this regard, in the specified period lobar pneumonia is characteristic.

Leukemia cells, infiltrating the interalveolar septa, as a rule, do not enter the lumen of the alveoli. In the respiratory region of the lung tissue, desquamation of the alveolar epithelium is observed, which further enhances exudation. In the absence of neutrophils, the alveolar macrophages play the main role in the clearance of alveoli from pathological contents. Therefore, the authors believe that the diagnosis of pneumonia in oncohematological patients is eligible even in cases of detecting serous contents in the alveoli in combination with the growth of fungal mycelium or microbial colonies.