[A case of hemolytic-uremic syndrome with development of catastrophic antiphospholipid syndrome: diagnosis and clinical tactics].

The paper describes a case of practically simultaneous development of the hemolytic-uremic syndrome (HUS) and the catastrophic antiphospholipid syndrome (CAPS) complicated by mesenteric vessel thrombosis and small bowel necrosis. Multimodality treatment comprising volume plasmapheresis, fresh frozen plasma transfusion, hemodialysis, anticoagulant and disaggregant therapy could relieve thrombogenic events, such as pulmonary artery thromboembolism and intestinal necrosis.

[Quantitative assessment of protein replacement in therapeutic plasmapheresis]. / Kolichestvennaia otsenka belkovogo zameshcheniia pri lechebnom plazmafereze.

AIM: To calculate parameters of replacement in which the amount of total protein (TP) in the circulating blood remains above critical level after removal of 17-75% of circulating plasm volume (CPL). MATERIAL AND METHODS: Therapeutic plasmapheresis (TPA) was made in 96 patients with rheumatoid arthritis, bronchial asthma, systemic lupus erythematosus and other diseases. The plasm was replaced by 0.9% sodium chloride solution, rheopolyglucine and albumin solutions in isovolemic regime. Albumin was given in quantities equivalent to 50-65% of the removed protein. RESULTS: Close correlation was observed between the expected and actual concentrations of total protein in 96 patients who have undergone 206 TPA procedures. In removal of up to 35% CPL protein deficiency is compensated by its mobilization from the deposits and due to protein-synthetizing function of the liver. In removal of 75% and more and in initial hypoproteinemia 50% of the protein should be replaced. CONCLUSION: It is confirmed that rheopolyglucin can be used for plasm replacement in TPA in patients with hyperproteinemia having no contraindications.

[Plasmapheresis in combine therapy of hypercoagulation syndrome in hematogenic thrombophilia]. / Plazmaferez v kompleksnoi terapii giperkoaguliatsionnogo sindroma pri gematogennykh trombofiliiakh.

AIM: To specify indications to plasmapheresis (PA) and to assess its efficiency in patients with hypercoagulatory syndrome in hematogenic thrombophilia (HT). MATERIAL AND METHODS: 18 patients (11 males, 7 females, age 26-50 years) with various forms of HT received standard antiaggregation, anticoagulatory therapy plus therapeutic PA. By PA technique the patients were divided into two groups. RESULTS: A positive trend in clinical, instrumental and laboratory indices was observed in all the patients. CONCLUSION: Therapeutic PA in hypercoagulation syndrome in HT patients leads to fast normalization of the clinical picture and reestablishment of patency of thrombus-affected vessels.

[Osmotic activity of plasma in plasmapheresis in patients with multiple myeloma]. / Osmoticheskaia aktivnost' plazmy pri plazmafereze u bol'nykh mnozhestvennoi mielomoi.

AIM: To control safety and efficiency of therapeutic plasmapheresis (PA) by osmolality, colloido-osmotic pressure (COP), total protein concentration before and after the procedure in patients with paraproteinemic hemoblastosis. MATERIAL AND METHODS: 20 patients with multiple myeloma have undergone 42 PA procedures conducted by two techniques: continuous flow centrifugation on blood fractioners or intermittent centrifugation of blood in plastic containers. The removed plasma volume averaged 1/3 (group 1) or 2/3 of the plasma volume (group 2). The removed protein reached 62-197 g. Isotonic sodium chloride solution and/or reopolyglucin (20-60 g) replaced the removed plasm. Total protein concentration was measured colorimetrically in biuretic reaction, plasma osmolality--cryoscopically and COP--on Knauer osmometer. RESULTS: PA leads to a short decline in osmolality (97.0-99.1%), of total protein concentration (82.8-78.6%) and of COD (79.2% in replacement with saline and 90.2% in replacement with dextran). During recovery after the procedure plasma osmotic activity and protein concentration return to the baseline. CONCLUSION: In elimination of 1/3 of plasma volume and crystalloid infusion, hemodilution promotes release of abnormal proteins from the tissues into the circulation and thereafter removal them from the organism. In removal of 1/2 and more of plasma volume, COP demans correction made by administration of colloids, e.g. solution of low molecular dextran. There is a potential danger of COD lowering several hours after PA due to different speed of dextran elimination and mobilization of protein reserve.

[Reestablishment of plasma proteins after plasmapheresis in patients with paraproteinemic hemoblastosis]. / Vosstanovlenie plasmennykh belkov posle plazmafereza u bol'nykh paraproteinemicheskimi gemoblastozami.

AIM: To study the effect of plasmapheresis (PA) on the changes in plasma total protein in the PA intervals in patients with paraproteinemic hemoblastosis (PPH). MATERIALS AND METHODS: 26 PPH patients underwent 80 PA procedures to remove macromolecular pathological proteins and diminish blood viscosity. Before PA the patients were examined hematologically, biochemically. Total protein concentration in plasma was measured before and after PA, in the removed plasma. RESULTS: PA promoted total protein lowering in plasma to 65-85% of the baseline irrespectively of PA technique. After PA the proteins gradually rose. Contents of circulation proteins were found unrelated to the number of PA procedures and averaged 70% of the PA-removed protein. CONCLUSION: The trends in recovery of plasma proteins after PA help to explain therapeutic effect in combination of PA with chemotherapy of paraproteinemic hemoblastoses.

[The monitoring of protein-volemic indices during plasmapheresis in patients with paraproteinemic hemoblastoses]. / Monitoring belkovo-volemicheskikh pokazatelei pri plazmafereze u bol'nykh paraproteinemicheskimi gemoblastozami.

We studied quantitative characteristics of plasma protein before, during and after 133 plasmapheresis (PA) procedures in patients with multiple myeloma and Waldenstrom's macroglobulinemia. A value of removed plasma volume (RPV) was calculated as a part plasma volume (PV) before PA with consideration of quantity and consequence of replacement solution. In the case when we removed 30% of calculated PV we replaced it only with electrolyte solutions. In the case of 50% PV removing, the replacement was a combination of low molecular weight dextran and electrolyte solutions (1:2) or 5% albumin and electrolyte solutions. The results support correlation between a level of total protein and RPV, kind of replacement solutions. We recommend two regression equations for efficient and safety planning RPV and for prediction of protein level after PA. This simple and fast method can be used for prognosis of critic PA parameters, to decrease a risk of side effects and for optimal use of albumin replacement solutions.

[Therapeutic plasmapheresis in immune complex diseases]. / Lechebnyi plazmaferez pri immunokompleksnykh zabolevaniiakh.

We studied the dependence of the plasma protein and volume on the exfusion volume, type and composition of the substitution solution in 140 plasmapheresis procedures given to patients with immune thrombocytopenic purpura and bronchial asthma. The change of blood protein system corresponded to the procedure regime. The procedure does not entail adverse reactions if it removes up to 30% of the plasma circulating volume (replacement with salt solution), 50% of the plasma circulating volume (replacement with dextran solution) or 95% of that with replacement with albumin. We have tested 3 simple mathematic models for postplasmapheresis proteinemia and recommend the regression equation Ct = a0 + a1.Co as the most adequate model.

[Plasmapheresis in the combined treatment of rheumatoid arthritis, complicated with anemia and blood coagulation disorders]. / Plazmaferez v kompleksnom lechenii revmatoidnogo artrita, oslozhnënnogo anemiei i narusheniem svërtyvaniia krovi.

Time course of changes in hemostasis were investigated in 68 rheumatoid arthritis (RA) patients. DIC syndrome and anemia were recorded in 23 and 20 patients, respectively. Three RA sufferers were free of anemia. A course of plasma-pheresis proved effective against DIC syndrome in 12 anemic and 2 nonanemic patients. Alleviation of DIC syndrome occurred in association with improvement in the red cell count and inhibition of rheumatoid process activity. In persistent DIC syndrome a trend to normalization of the above parameters was insignificant.

[Comparative assessment of various programs of plasmapheresis in the combined treatment of patients with rheumatoid arthritis]. / Sravnitel'naia otsenka razlichnykh programm plazmafereza v kompleksnom lechenii bol'nykh revmatoidnym artritom.

Therapeutic efficacy of various plasmapheresis (PA) programmes was comparatively studied in 92 patients with rheumatoid arthritis. PA performed as a placebo (the control group) confirmed the efficacy of PA in the patients with rheumatoid arthritis. The choice of a particular regimen of the procedure depended on the health status of the patient. The programme of PA with the elimination of one volume of the circulating plasma (VCP) turned to be the most effective in the patients with highly active course of the disease while the procedures with the elimination of 1/2 and 1/4 VCP should be used in those with moderate or minimal activity of the disease. The assessment of the therapeutic response should be based on the clinical data, ESR and the contents of circulating immune complexes--the most dynamic laboratory tests.

[Blood protein-volume indicators in massive plasmapheresis]. / Belkovo-volemicheskie pokazateli pri massivnom plazmafereze.

Hyperproteinemia values were studied before and after plasmapheresis in 32 rheumatoid arthritis patients with no manifest circulatory disorders. Mathematical patterns enabling prediction of changes in protein concentration depending on the volume of removed plasma and the type of substitution have been proposed for 3 alternative regimens of the procedure (removal of 0.5 or 1.0 volume of the circulating plasma, and substitution for rheopolyglucin or albumin). The data of independent "examining" sampling (15 patients) have proved adequacy of the linear regression patterns.

[Colloid osmotic pressure and the total protein level in assessing the safety of therapeutic plasmapheresis]. / Kolloidno-osmoticheskoe davlenie i uroven' obshchego belka v otsenke bezopasnosti lechebnogo plazmafereza.

In 117 plasmapheresis procedures provided to rheumatic arthritis patients, the authors studied the changes in colloid osmotic pressure (COP), proteinemia and systemic arterial pressure depending on the plasma volume removed and on the type and volume of the substituting solution. Plasmapheresis carried out in 3 alternative regimens was not accompanied by dangerous exchange in the protein system. The substitution of the removed plasma by albumin and rheopolyglukin ensures an effective maintenance of COP just after the procedure. Since the elimination of polyglucin from the vascular system proceeds quicker than making up for protein deficiency, there is a potential danger of inadequate reduction of COP several hours after the procedure. Simple mathematic models have been proposed for the description of the COP level and proteinemia after plasmapheresis, which can be used for prognosis.

[Experience with the use of plasmapheresis in the combined treatment of children with the crush syndrome]. / Opyt primeneniia plazmafereza v kompleksnom lechenii detei s sindromom dlitel'nogo sdavleniia.

Plasmapheresis (PP) was applied to the treatment of 5 children aged 6-14 years with the crush syndrome. The multimodality treatment using antibiotics, erythrocytic mass, rheologically active preparations, hemodialysis and blood rheologically active preparations, hemodialysis and hemoperfusion carried out for 6-7 days before PP did not bring about any appreciable improvement of the patients' status. The patients failed to get rid of anuria and manifested the signs of increasing intoxication. The treatment with PP consisted of 1 to 6 procedures, in the course of which 70 to 85 of the design volume of the circulating plasma was removed. The use of PP resulted in the disappearance of myoglobin from the patients' blood and urine, in the normalization of the coagulogram, a considerable decrease of the content of medium molecules, and in the appearance of the first urine towards the end of the procedure. Thus, the introduction of PP into multimodality treatment of the crush syndrome made it possible to eliminate anuria, disseminated intravascular coagulation, and to noticeably reduce intoxication and to clear the blood off myoglobin.

[The protein-blood volume indices during moderate plasmapheresis]. / Belkovo-volemicheskie pokazateli pri umerennom plazmafereze.

Quantitative characteristics of changes in the volume of circulating plasma and the amount of circulating protein were studied in rheumatoid arthritis patients before and after plasmapheresis of moderate intensity. Basing on the comparison of the precalculated and estimated parameters the authors have suggested 4 mathematical models equally describing the protein-volemic relationships in the body. Satisfactory correlation has been recorded between the estimated and precalculated values of protein concentration in the plasma after plasmapheresis that enables data collection in a short cut program.

[Immunocorrective treatment of idiopathic thrombocytopenic purpura by plasmapheresis]. / Immunokorrigiruiushchee lechenie idiopaticheskoi trombotsitopenicheskoi purpury putem plazmafereza.

Patients with idiopathic thrombocytopenic purpura (ITP) tolerated well plasmapheresis. In no cases hemorrhage intensification was observed. An immediate clinical effect was recorded in all patients that correlated with the immunological parameters improvement in most of them. The platelet count significantly rose only in one patient immediately after the plasmapheresis course. The follow-up of the patients during 11-20 months has enabled revealing not only immediate clinical but also late hematological effect of plasmapheresis, that was manifest in a delayed rise of the platelet levels in most patients, and abolished resistance to glucocorticoids in one of them. It has been concluded that plasmapheresis produces immunocorrecting effect and could be used for the treatment of ITP patients.