afibrinogenemia

afibrinogenemia /afi·brin·o·gen·emia/ (a″fi-brin″o-jĕ-ne´me-ah) deficiency or absence of fibrinogen (coagulation factor I) in the blood.

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congenital afibrinogenemia  a rare autosomal recessive hemorrhagic coagulation disorder characterized by complete incoagulability of the blood.

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a·fi·brin·o·ge·ne·mi·a (f-brn-j-nm-, -fbr-n-)

n.

The absence of fibrinogen in the blood plasma.

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afibrinogenemia

[afī′brinō′jenē′mē·ə]

Etymology: Gk, a, not; L, fibra, fiber; Gk, genein, to produce, haima, blood

a relative lack or absence of fibrinogen in the blood. It may be the result of a primary, congenital blood dyscrasia, or it may be acquired, as in disseminated intravascular coagulation. Also spelled afibrinogenaemia.

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afibrinogenemia [a″fi-brin″o-jĕ-ne´me-ah]

absence or deficiency of fibrinogen in the circulating blood. Congenital afibrinogenemia (complete absence of fibrinogen) is a rare anomaly that is inherited. Acquired afibrinogenemia is actually a deficiency of fibrinogen (hypofibrinogenemia) and often is a serious complication in obstetrics, the primary cause being excessive maternal use of fibrinogen during an abnormal pregnancy. The condition may be seen in association with malignancies of the bone and prostate and with leukemia. It also may follow transfusion of incompatible blood and sometimes may complicate thoracic and abdominal surgery.

Symptoms. As would be expected in a deficiency of fibrinogen, which plays an important role in the blood clotting mechanism, the chief symptom is generalized bleeding, external or internal. In obstetric or surgical patients suffering from this condition there is frequently sudden and uncontrollable hemorrhage.

Treatment. Fibrinogen is administered intravenously to supply the body with this essential substance; transfusions of fresh frozen plasma or cryoprecipitate may also be indicated. In patients with cancer of the prostate the fibrinogen level often returns to normal after administration of estrogens. In obstetric patients the fibrinogen level returns to normal after the uterus has been emptied.

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afibrinogenemia

absence or deficiency of fibrinogen in the circulating blood. The defect is inherited in goats, causing a severe hemorrhagic diathesis. See also hypofibrinogenemia, dysfibrinogenemia.

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afibrinogenemia

Hematology A rare AR condition characterized by complete incoagulability of blood, which may be first identified by excess bleeding from the umbilical stump; other findings include bone and liver lesions, and spontaneous rupture of spleen