ABSTRACT
Methodological recommendations for surgical care in patients with hemophilia A receiving prophylactic therapy with emicizumab. Recommendations of the expert group. Moscow, 2024.
Subject(s)
Antibodies, Bispecific , Antibodies, Monoclonal, Humanized , Hemophilia A , Humans , Hemophilia A/complications , Hemophilia A/drug therapy , Factor VIII , Hemorrhage/prevention & control , Antibodies, Bispecific/adverse effectsABSTRACT
We measured specific volume and hematocrit of blood clots prepared from the whole blood of patients with hemophilia A and healthy male volunteers. It was shown that in the hematocrit range of 43.5-52.5%, specific volume of the blood clot in hemophilia patients with low level of factor VIII (1-4%) was higher than in volunteers. After injection of factor VIII, specific volume of blood clots in hemophilia patients decreased. Hematocrit of the blood clots derived from the whole blood linearly depended on the mean erythrocyte density in both volunteers (r=-0.74, p=0.01) and patients with factor VIII level of 1-4% (r=-0.95, p<0.0001). The increase in the mean erythrocyte density led to a decrease in blood clot hematocrit. The curve describing blood clot hematocrit as a function of the mean erythrocyte density in hemophilia patients was lower than in healthy volunteers. The increase in factor VIII level was associated with an increase in blood clot hematocrit. The results showed that blood clot hematocrit depends on the mean erythrocyte density, and therefore, hematocrit of the blood clot can be changed by modulating the properties of erythrocyte population.
Subject(s)
Blood Coagulation , Hemophilia A/blood , Thrombosis/blood , Adult , Blood Coagulation Tests , Case-Control Studies , Erythrocyte Indices , Erythrocytes/metabolism , Erythrocytes/pathology , Factor VIII/metabolism , Hematocrit , Hemophilia A/physiopathology , Humans , Male , Middle Aged , Thrombosis/physiopathologyABSTRACT
The paper describes 4 clinical cases of thrombotic events (pulmonary embolism, deep vein thrombophlebitis, acute myocardial infarction, ischemic stroke) that have occurred in patients with hemophilia. It discusses the possible causes of their development and methods for their prevention and treatment. Controlled natural hypocoagulation, in which the dose of an administered deficient factor decreases to such an extent that in order to maintain the safe level of hypocoagulation (plasma factor activity is 15-20%; activated partial thromboplastin time is 1.5-2 times normal values), is proposed as one of the treatment options.
Subject(s)
Brain Ischemia , Factor VIII , Hemophilia A , Myocardial Infarction , Pulmonary Embolism , Stroke , Thrombophlebitis , Adult , Blood Coagulation/physiology , Blood Coagulation Tests/methods , Brain Ischemia/etiology , Disease Management , Factor VIII/administration & dosage , Factor VIII/analysis , Hemophilia A/complications , Hemophilia A/diagnosis , Hemophilia A/physiopathology , Hemophilia A/therapy , Humans , Male , Middle Aged , Myocardial Infarction/diagnosis , Myocardial Infarction/etiology , Myocardial Infarction/physiopathology , Myocardial Infarction/therapy , Pulmonary Embolism/diagnosis , Pulmonary Embolism/etiology , Pulmonary Embolism/physiopathology , Pulmonary Embolism/therapy , Stroke/diagnosis , Stroke/etiology , Stroke/physiopathology , Stroke/therapy , Thrombelastography/methods , Thrombophlebitis/diagnosis , Thrombophlebitis/etiology , Thrombophlebitis/physiopathology , Thrombophlebitis/therapy , Treatment OutcomeABSTRACT
We examined HCV+ and HCV- hemophilia A patients with knee arthropathy and hematocrit above 38.5%. The mean density of erythrocytes was studied by the phthalate method, intraoperative blood loss was assessed gravimetrically. The volume of blood loss in HCV+ patients with manifest adhesive process and chronic synovitis varied from 300 to 1900 ml, in patients with moderate adhesive process from 400 to 1500 ml. The volume of blood loss in HCV- patients was 300-800 ml. A positive correlation between the blood loss volume and mean density of erythrocytes was detected. Blood loss >1000 ml during total knee arthroplasty can be expected in patients with hemophilia A with HCV and high mean density of erythrocytes. Blood loss >1000 ml is unlikely in HCV- and HCV+ patients with the mean density of erythrocytes not surpassing the normal values.
Subject(s)
Blood Loss, Surgical , Hemophilia A/surgery , Hepatitis C/surgery , Osteoarthritis, Knee/surgery , Adult , Arthroplasty, Replacement, Knee , Blood Volume , Hemophilia A/pathology , Hepatitis C/pathology , Humans , Middle Aged , Osteoarthritis, Knee/pathology , Young AdultABSTRACT
Intraoperative blood loss during total knee arthroplasty in patients with hemophilia varies over a wide range (from 300 to 3000 ml). The reasons have not been clarified yet. We studied the dependence of intraoperative blood loss during total knee arthroplasty in patients with hemophilia A on hematocrit and mean erythrocyte density. Intraoperational blood loss ≥1000 ml was observed in patients with hematocrit <38.5%. In patients with hematocrit >38.5% this parameter depended on the mean erythrocyte density: in patients with increased erythrocyte density, the risk of intraoperational blood loss ≥1000 ml was higher. The increase in erythrocyte density can serve as an indicator of pathological processes, including the processes modulating hemostasis. It can also be assumed that erythrocytes with higher density change blood flow, which affects platelet adhesion to the damaged endothelium. Hematocrit below the threshold level and mean density of erythrocytes above the normal level can be regarded as risk factor for increased intraoperational blood loss.
Subject(s)
Arthroplasty, Replacement, Knee/adverse effects , Blood Loss, Surgical/physiopathology , Erythrocytes/physiology , Hematocrit , Hemophilia A/surgery , Female , Hemophilia A/physiopathology , Humans , MaleABSTRACT
Hairy cell leukemia (HCL), a chronic B-cell lymphoproliferative disease with special villous morphology and immunophenotypic markers of lymphoid cells, is characterized by the involvement of bone marrow and spleen. The paper describes a case of a 29-year-old female patient without abnormal clinical blood tests and myelograms, with normal spleen sizes, in whom the only manifestation of HCL was massive scrotal injury with a soft tissue component in the small pelvic cavity.