Afibrinogenemia

Afibrinogenemia is the absence in the blood fibrinoguena; decrease of its content in blood - fibrinoguena (gipofibrinogenemia).
If afibrinogenemia coagulation occurs, when fibrinogenous it slowed down, formed a loose or krokovay clot. Blood clotting is terminated when the plasma is less than 60 mg% of fibrinogen (normally the content of fibrinogen plasma is 300 to 500 mg%).
Afibrinogenemia may be congenital (very rarely) and acquired. With the innate afibrinogenemia any trauma to the child leads to bleeding, and with the beginning walk appear ekhimozy, hematoma, hemorrhage in the joint cavity. The diagnosis of afibrinogenemia put upon detection full nesvertawateisa blood and absence of fibrinogen plasma under normal results of other samples of hemorrhagic syndrome (negative symptom harness, bleeding time and retraction of the clot normal number of platelets and prothrombin index is not changed). Children with congenital afibrinogenemia rarely live to a ripe age.
Acquired afibrinogenemia can depend both on the lack of education of fibrinogen in the defeat of the authorities where it is produced (liver, lungs, bone marrow), and from increased its dissolution of fibrinolysis. In the first case is often fibrinoguena, which is echinosoma, haematoma formation in the slightest trauma, hematuria. Coagulation occurs, but in a few minutes or hours, the blood becomes liquid. Such picture is observed with cirrhosis of the liver and acute necrosis her, when prostate cancer or pancreatic cancer with metastases in bone marrow, with acute leukemia. In less severe form of fibrinoguena marked sometimes by poligonului, pancytopenia, chronic myeloid leukemia. In these cases, formed a blood clot does not dissolve, but crumbles easily.
In the case of enhanced fibrinolysis develops acute afibrinogenemia observed sometimes in shock of any origin (traumas, burns, acute hemolysis, asphyxia), and in complicated childbirth (fetal death of the fetus, premature detachment or manual separation of the placenta, getting the blood of amniotic fluid). In addition to enhanced fibrinolysis, the cause of acute afibrinogenemia in these cases, it may be intravascular coagulation with power consumption of fibrinogen under the influence of penetration into blood thromboplastin from the placenta and amniotic fluid. Coming this indomitable uterine bleeding quite often forces to resort to urgent removal of the uterus. Similar afibrinogenemia observed rarely after operations on the lungs and pancreas. Such cases require urgent diagnostics, the main element of which is the discovery of the absence of fibrinogen. Often such patients the blood not only not folded, but dissolves the clot normal blood.
The main treatment of afibrinogenemia of any kind is (after eliminating, where possible, the reasons for it) transfusion of large doses of the blood (2-3 l) or plasma (1-2 l), and intravenous introduction of fibrinogen (2-5 g).

Afibrinogenemia - the complete absence in the plasma of one of its proteins, fibrinogen. Afibrinogenemia, usually congenital and is extremely rare.
Often there is gipofibrinogenemia - reduction fibrinoguena content in plasma, sometimes inborn and usually acquired character. Acquired gipofibrinogenemia may be the result of inadequate education of fibrinogen in the liver (hepatitis, cirrhosis, different types of protein depletion); intensive use of fibrinogen by the release into the bloodstream tromboplastina active substances (intravascular hemolysis, fetal death of the fetus, previa and placental abruption); increased activity fibrinoliticescoy blood system in shock, detachment of the placenta, prostate and pancreas and operations on these bodies, erythremia, chronic myelogenous leukemia and other
For afibrinogenemia characterized by the absence of whole blood coagulation, even when you add to it thrombin or thromboplastin, and the absence of fibrinogen when quantifying; if gipofibrinogenemia clotting sharply slowed, the content of fibrinogen reduced.
The pathogenesis of congenital A. and gipofibrinogenemia not installed. There is an assumption about hereditary character, and A. complies with homozygous and gipofibrinogenemia - heterozygous state.
Clinical manifestations of afibrinogenemia limited to bleeding, mainly after traumas, operations and other external influences, sometimes from the very first days of life.
Rare spontaneous hemorrhage (from gums, nose, and others) and hemarthrosis. If gipofibrinogenemia the intensity of bleeding is greater, the lower fibrinoguena level in plasma. With a slight lack of fibrinogen bleeding may be missing. Symptomatic gipofibrinogenemia with liver disease is an important diagnostic symptom, reflecting the functional state of the body. Expressed gipofibrinogenemia and especially A. if the liver adverse prognostic: they point to the development of dystrophy.
Treatment of afibrinogenemia reduced to stop bleeding by means of the preparation of fibrinogen in the dose of 5-8 g or appropriate quantity of plasma (100 ml plasma contain 0.2-0.3 g fibrinoguena). The bleeding stops when reaching the fibrinoguena level in plasma of approximately 80 mg%. With acquired forms an important treatment of the underlying disease. It is necessary to consider, that during massive blood loss due to bleeding in labor abundant transfusion citrate blood can cause the development of gipofibrinogenemia or enforce it. Cm. also the Blood (physico-chemical properties).