[Generalized immune-complex vasculitis combined with pulmonary hemosiderosis and dilated cardiomyopathy]. / Generalizovannyi immunokompleksnyi vaskulit v sochetanii s legochnym gemosiderozom i dilatatsionnoi kardiomiopatiei.

The paper is concerned with observations over 3 patients in whom unusual vasculitis lay at the basis of the clinicopathological manifestations. All the patients were men of the young age. The disease debut was marked by fever, weakness, dyspnea, palpitation, cough, hemoptysis, the articulation syndrome. In two cases, there was hemorrhagic rash on the leg skin. All the patients manifested liver and spleen enlargement, two patients had lymphoadenopathy. The leading clinical symptoms included dilated cardiomyopathy, complete blockade of the inferior peduncle of His bundle and reduction of myocardial contractility. Anemia belonged to iron deficient one. The clinical examples provided indicate that immunocomplex vasculitis with evident lesions of the lungs and myocardium, not going into criteria for the known diseases, is not likely to be a casuistic rarity. Those syndromes may be associated with more or less pronounced hemosiderosis of the lungs (and, probably, of the lymph nodes, spleen and liver), with transitory or steady derangements of myocardial conduction, which attests to diffuse lesions of the myocardium possibly with both immune complexes and hemosiderin. The pathology requires further studies.

[The problems of plasma substitution in performing therapeutic plasmapheresis (an evaluation of the efficacy of plasma substitute media and the methods for obtaining them)]. / Problemy plazmozameshcheniia pri provedenii lechebnogo plazmafereza (otsenka éffektivnosti plazmozameshchaiushchikh sred i metody ikh polucheniia).

The analysis of 3700 plasmaphereses (AP) performed in 692 patients permitted the authors to develop the basic principles and policy of plasma substitution for various groups of patients. The use of protein preparations and fresh frozen plasma is believed essential in intensive plasmapheresis in patients with immunomediated disorders and DIC syndrome, respectively. Potentialities of selective therapeutic PA are discussed. PA criteria should be based on the patient's state, therapeutic aims, circulation parameters, laboratory data.

[The results of 5 years' use of a method for heparin cryoprecipitation of plasma proteins (selective plasmapheresis) in patients with an immune-complex pathology]. / Rezul'taty 5-letnego primeneniia metoda kriogeparinopretsipitatsii plazmennykh belkov (selektivnogo plazmafereza) u bol'nykh s immunokompleksnoi patologiei.

The paper treats the results of the 5-year use of plasma protein heparin cryoprecipitation (selective plasmapheresis) (SPA) in patients with immune complex pathology. The method consists in the removal of fibronectin and bound complement components, cryoglobulins, cryofibrinogen, circulating immune complexes (CIC), and other gross-dispersed proteins in the presence of heparin in the cold. The SPA was applied to 122 patients with immune complex diseases and syndromes. Altogether 1279 procedures were carried out. The method turned out effective in 80% of patients with hemorrhagic vasculitis, systemic lupus erythematosus, rheumatoid arthritis, and other types of vasculitis. The changes in the blood serum concentration of immune complexes correlated with the time-course of changes in the clinical and laboratory parameters. The SPA efficacy was in agreement with the reduction in the level of immune complexes. Provided 2-3 procedures were carried out a week, the lowering of the level of immune complexes and other laboratory parameters marking the degree of inflammation could be seen by procedures 4-6. Therefore, to attain a stable clinical effect, its is necessary to perform not less than 6 SPA procedures. It has been demonstrated that SPA can be used an unlimited number of times, for the use of the method does not entail the development of plasma protein deficiency. This circumstance is of paramount importance for the treatment of chronic relapsing immune complex diseases.

[Apheresis of euglobulins, cryoglobulins and the von Willebrand factor in vasculitis]. / Aferez éuglobulinov, krioglobulinov, faktora Villebranda pri vaskulitakh.

A method of apheresis of plasma euglobulin fraction, cryoglobulins and Willebrand factor was developed. In one session of plasmapheresis 1500-1700 ml of patient's plasma were removed, fractionated and returned. The method was used in 2 patients with immune complex vasculitides. In one of them the disease developed against a background of chronic active hepatitis, in the other patient it manifested itself in cryoglobulin- and cryofibrinogenemia. Clinical improvement was noted in both cases: the absence of myalgia, arthralgia, hemorrhagic eruption, and ulcerative-necrotic skin changes. The normalization of increased ristomycin-cofactor activity of Willebrand factor and CIC levels was noted in one case. A decrease in the content of plasma cryoglobulins, cryofibrinogen, and urine protein concentration (from 1.5 up to 0.03%) was noted in the other case. A possibility of the use of the method in other pathological conditions (DIC-syndrome, unstable angina, atherosclerotic angiopathy) accompanied by endothelial damage, was discussed. Willebrand factor multimers form complexes with low density lipoproteins therefore the removal of these complexes may be useful in the treatment of hypercholesteremia and atherosclerosis.

[Effect of cryoapheresis on the course of immune complex diseases]. / Vliianie krioafereza na techenie immunokompleksnykh zabolevanii.

The paper is concerned with an analysis of the results of the method of cryoheparinoprecipitation of plasma proteins in patients with immune complex pathology. The essence of the method is that in plasma cooling cryofibrinogens, cryoglobulins, immune complexes and other components of the complement system aggregate and precipitate. The process is potentiated by adding heparin to the plasma. After removal of cryoproteins the plasma is frozen and returned to a patient during a session of plasmapheresis. The method makes it possible to give up the use of various protein preparations as substitutes. It was applied to 90 patients: 52 females and 38 males aged 15 to 64. The total number of plasmapheresis sessions was 831, an average of 6 sessions per patient; repeated sessions were performed in 20 patients in different time-intervals. Indications for therapy were the absence of a response to routine drug therapeutic methods for a long period of time and steroid-related complications. Cryoapheresis was applied to 18 patients with hemorrhagic vasculitis. Signs of the abdominal, dermal and articular syndrome were eliminated in all the patients; the urinary syndrome was retained in half of the cases necessitating other therapeutic modalities. A CIC level investigated by a method of precipitation with 3.5% polyethylene glycol was not a criterion of assessment of the gravity of diseases: in cases of its increase before therapy initiation there was a clear tendency to a decrease and normalization by the end of treatment. In hemostasis investigation hypercoagulation activation and hyperfibrinogenemia were revealed in most of the patients.(ABSTRACT TRUNCATED AT 250 WORDS)

[Plasma fibronectin level in patients with depression of hematopoiesis]. / Kontsentratsiia plazmennogo fibronektina u bol'nykh s depressiiami krovetvoreniia.

Plasma fibronectin is regarded to play an important part in a decrease of the resistance to infections. To specify the role of fibronectin in the pathogenesis of infectious complications in patients with depressions of hemopoiesis, the content of this opsonin was measured by ELISA in 113 patients with different patterns of hemoblastoses, lymphoproliferative diseases and with an aplastic syndrome. In 42 patients, the concentration of opsonin was measured in the presence of the superimposed infection of varying gravity. The fibronectin content was examined in 39 patients before, during and after completion of the cytostatic polychemotherapy. It turned out that in patients with paraproteinemic hemoblastoses, lymphogranulomatosis, aplastic anemia, chronic lympholeukemia, acute lympho- and myelo(mono)blastic leukemias, cyclic neutropenia, chronic myelosis and hematosarcomas, the concentration of fibronectin remained normal in the absence of infections. The computation of the linear correlation ratio did not reveal any association between the opsonin level and the concentration of neoplastic elements in the peripheral blood. Repeated measurements of the fibronectin level in patients whose underlying disease ran its course in association with marked neoplastic fever failed to detect any deficiency of the glycoprotein. The lowering of the fibronectin level was recorded in patients with a grave concomitant infection of the type of sepsis, necrotic enteropathy and lobar pneumonia. The degree of opsonin deficiency correlated with the patients' disease gravity. Prolonged reduction in the blood fibronectin level was of unfavourable prognostic importance. Cytostatic polychemotherapy, myelotoxic agranulocytosis as well as infectious complications of low gravity did not influence the concentration of fibronectin.(ABSTRACT TRUNCATED AT 250 WORDS)

[Effect of the method of extracorporeal heparin precipitation of plasma proteins (selective plasmapheresis) on the level of immune complexes in the blood]. / Vliianie metoda ekstrakorporel'noi geparinopretsipitatsii plazmennykh belkov (selektivnogo plazmafereza) na kontsentratsiiu immunnykh kompleksov v krovi.

A study was made of the effect of selective plasmapheresis (SPP) on the concentration of circulating immune complexes (CIC). The method is based on precipitation at 4 degrees C of plasma fibronectin and associated macromolecular complexes by means of heparin. Sterile plasma of the patient is separated from the precipitate, frozen and kept at -20 degrees C till the next plasmapheresis during which it is returned to the patient instead of the donor's one. All 15 patients examined were exposed to 6 SPP with an interval made every 2 to 5 days. Six patients were diagnosed to have rheumatoid arthritis, 2 systemic lupus erythematosus, 3 hemorrhagic microthrombovasculitis, and 4 multiple sclerosis. The concentration of CIC was measured by precipitation with 3.5% polyethylene glycol before and after SPP, in some cases between sessions. All the patients with rheumatoid arthritis and systemic lupus erythematosus and 2 out of the 3 patients with hemorrhagic vasculitis showed an elevated content of CIC (greater than 0.150 Units OD). The CIC content appeared normal in all the patients with multiple sclerosis. After SPP 4 patients manifested a reduction in the CIC concentration, whereas in 6 it returned to normal. Such a time course correlated with the improvement of other clinical and laboratory findings. It was established that after the first session of SPP the CIC content sharply declined followed by a gradual increment and exceeded the initial values toward the beginning of the second session. After the second SPP the patients manifested the same tendencies. The CIC content reached a maximum by the third SPP and then fell from session to session.(ABSTRACT TRUNCATED AT 250 WORDS)

[Clinical effectiveness of the method of extracorporeal heparin precipitation of plasma proteins (selective plasmapheresis) in patients with immune complex pathology]. / Klinicheskaia effektivnost' metoda ekstrakorporal'noi geparinopretsipitatsii plazmennykh belkov (selektivnogo plazmafereza) u bol'nykh s immunokompleksnoi patologiei.

The authors analyse the efficacy of extracorporeal heparin precipitation of plasma proteins (selective plasmapheresis) in patients with immune complex pathology. The method lies in extracorporeal precipitation at +4 degrees C of fibronectin, cryofibrinogen and other macromolecular complexes with the aid of heparin, followed by plasma return to the patient. The method was applied to 39 patients. Hemorrhagic microthrombovasculitis was diagnosed in 7, vasculitis paralleled by hepatosplenomegaly of unclear genesis in 1, generalized vasculitis with marked systemic manifestations in 1, immune complex vasculitis complicated by recurrent polychondritis in 1, chronic active hepatitis associated with immune complex vasculitis in 1, rheumatoid arthritis in 8, systemic lupus erythematosus in 4, spleen lymphoma and Sjögren's syndrome in 1, idiopathic amyloidosis in 2, multiple sclerosis in 10 patients. Altogether 309 sessions of selective plasmaphapheresis were carried out. The time course of the clinical and laboratory criteria of the disease activity were studied before and after each plasmapheresis session. It was ascertained that the method is highly effective in the treatment of hemorrhagic vasculitis. It is mostly used if conventional therapy with heparin, cryoplasm, and deaggregating agents appears abortive. Apparently, it is the only method that may rapidly arrest the abdominal syndrome associated with hemorrhagic vasculitis. The possibility and advisability of the use of chronic plasmapheresis are discussed. Application of the method to the treatment of rheumatoid arthritis permits reducing the dose of steroid hormones, thereby not stimulating the disease activity. The method is the therapy of choice in different diseases associated with polyvalent allergy. In amyloidosis and multiple sclerosis, the method efficacy is questionable.(ABSTRACT TRUNCATED AT 250 WORDS)