In the present study, patients in the lowest quartile of C1-esterase inhibitor activity in combination with high C-reactive protein demonstrated a higher risk of developing severe sepsis. In general, high-dose C1-esterase inhibitor infusion down-regulated the systemic inflammatory response and was associated with improved survival rates in sepsis patients, which could have important treatment and survival implications for individuals with C1-esterase inhibitor functional deficiency.

CO measured by COstatus was found to be equivalent and hence interchangeable with PiCCO in this study population. COstatus blood volumes were found to be within the expected physiological range whilst PiCCO blood volumes were significantly higher, which was also observed in other studies. Future studies using 3D echo/MRI are required to validate these blood volumes.

Purpose: To investigate the safety of the of bone marrow-derived human multipotent mesenchymal stromal cells (MMSCs) administration in neutropenic patients with septic shock. Methods: A single center prospective randomized Russian clinical trial of MMSCs in neutropenic patients with septic shock was registered (RUMСESS) (NCT 01849237). The patients were randomly assigned to receive conventional therapy (CT) (n = 15) or intravenous MMSCs + CT (n = 15) at the dose of 1 × 10 6 MMSCs/kg. Results: Initial SOFA scores were 15.9 (95% CI 13.9-20.2) in MMSCs + CT and 16.1 (95% CI 12.1-19.0) in CT group. For second day cardiovascular component of SOFA and PaO 2 /FiO 2 improved in MMSC + CT group. 28-days survival rates were 20% (3/15 patients) in CT group and 60% (9/15 patients) in the MMSC + CT group (P < 0.05). In MMSC + CT group only 4 patients remained alive in 3 months, 5 patients died due to the infection related organ dysfunctions, in CT group 3 patients remained alive in 3 months. Conclusions: Administration of MMSCs in neutropenic patients with septic shock was well tolerated and attributed to the faster hemodynamic stabilization, vasopressor withdrawal, attenuation of respiratory failure and shortening of the neutropenia duration period.

No abstract

A lower chemotherapeutic load and a small number of allogeneic BMTs did not affect total positive treatment results in adult patients with ALL, by complying with the principle achieving the continuity of cytostatic effects and by preserving the total cytostatic loading dose. The results of the Russian investigation casts some doubt on the necessity of using very intensive consolidation cycles and performing a large number of allogeneic BMTs in adult patients with ALL.

Hemostatic disorders play an important role in the pathogenesis and clinical manifestations of COVID-19. The purpose of the research was a detailed consideration of the pathogenesis, clinical manifestations, and methods of diagnosing and treatment of coronavirus-induced coagulopathy (CIC). At the onset of COVID-19, hypercoagulability is detected, and consumption coagulopathy and disseminated intravascular coagulation (DIC) syndrome are usually observed at later stages of the disease. In the pathogenesis of hypercoagulation in patients with COVID-19, proinflammatory cytokines, hyperfibrinogenemia, increased blood levels of von Willebrand factor, factor VIII, neutrophilic extracellular traps, platelet activation, production of antiphospholipid antibodies, microvesicles are of importance. Laboratory findings show increased plasma concentrations of D-dimer, fibrinogen, a longer prothrombin time and a decrease in the number of platelets. The cumulative incidence of thrombotic complications ranges from 21 to 31%. Thrombosis risk factors are intensive care unit stay, leukocytosis, and a high plasma D-dimer concentration. Differential diagnosing of CIC should be carried out with disseminated intravascular coagulation, sepsis-induced coagulopathy, antiphospholipid, hemophagocytic syndromes, thrombotic microangiopathy, and heparin-induced thromocytopenia. CIC may be complicated by sepsis, antiphospholipid syndrome, hemophagocytic syndrome, thrombotic microangiopathy, and heparin-induced thrombocytopenia.The main therapy is low molecular weight heparins treatment. Treatment recommendations are provided.

No abstract

Gaucher disease is the most common form of hereditary enzymopathies combined into a group of lysosomal storage diseases. The basis for the disease is a hereditary deficiency of the activity of acid β-glucosidase, a lysosomal enzyme involved in the catabolism of lipids, which results in the accumulation of nonutilized cellular metabolism products in the macrophage lysosomes. The main clinical manifestations of type 1 Gaucher disease are cytopenia, hepatomegaly, and splenomegaly, and bone lesion. One of the atypical clinical manifestations of Gaucher disease is damage to the lungs with the development of pulmonary hypertension, which is usually considered within the underlying disease - the development of pneumosclerosis due to macrophage dysfunction. The paper describes a case of progressive pulmonary hypertension in a patient with type 1 Gaucher disease.