Canadian Medical Guide > Diseases > Hemic and Lymphatic Diseases > Hematologic Diseases > Blood Coagulation Disorders > Coagulation Protein Disorders Terms and Definitions




Coagulation Protein Disorders

Medical Definition: Hemorrhagic and thrombotic disorders resulting from abnormalities or deficiencies of coagulation proteins.
Guide Notes: GEN: prefer specifics; coord IM with coag factor (IM)
Previously Indexed: Blood Coagulation Disorders (1966-1998)
Coagulation Protein Disorders Categories.
Canadian Medical Guide Definition Activated Protein C Resistance - A hemostatic disorder characterized by a poor anticoagulant response to activated protein C (APC). The activated form of Factor V (Factor Va) is more slowly degraded by activated protein C. Factor V Leiden mutation (R506Q) is the most common cause of APC resistance.
Canadian Medical Guide Definition Afibrinogenemia - A deficiency or absence of fibrinogen (coagulation factor I) in the blood. (Dorland, 27th ed)
Canadian Medical Guide Definition Factor V Deficiency - A deficiency of blood coagulation factor V (known as proaccelerin or accelerator globulin or labile factor) leading to a rare hemorrhagic tendency known as Owren's disease or parahemophilia. It varies greatly in severity. Factor V deficiency is an autosomal recessive trait. (Dorland, 27th ed)
Canadian Medical Guide Definition Factor VII Deficiency - An autosomal recessive characteristic or a coagulation disorder acquired in association with VITAMIN K DEFICIENCY. FACTOR VII is a Vitamin K dependent glycoprotein essential to the extrinsic pathway of coagulation.
Canadian Medical Guide Definition Factor X Deficiency - Blood coagulation disorder usually inherited as an autosomal recessive trait, though it can be acquired. It is characterized by defective activity in both the intrinsic and extrinsic pathways, impaired thromboplastin time, and impaired prothrombin consumption.
Canadian Medical Guide Definition Factor XI Deficiency - A deficiency of blood coagulation factor XI (known as plasma thromboplastin antecedent or PTA or antihemophilic factor C) resulting in a systemic blood-clotting defect called hemophilia C or Rosenthal's syndrome, that may resemble classical hemophilia. (Dorland, 27th ed)
Canadian Medical Guide Definition Factor XII Deficiency - An absence or reduced level of blood coagulation factor XII. It normally occurs in the absence of patient or family history of hemorrhagic disorders and is marked by prolonged clotting time.
Canadian Medical Guide Definition Factor XIII Deficiency - A deficiency of blood coagulation FACTOR XIII or fibrin stabilizing factor (FSF) that prevents blood clot formation and results in a clinical hemorrhagic diathesis.
Canadian Medical Guide Definition Hemophilia A - The classic hemophilia resulting from a deficiency of factor VIII. It is an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage.
Canadian Medical Guide Definition Hemophilia B - A deficiency of blood coagulation factor IX inherited as an X-linked disorder. (Also known as Christmas Disease, after the first patient studied in detail, not the holy day.) Historical and clinical features resemble those in classic hemophilia (HEMOPHILIA A), but patients present with fewer symptoms. Severity of bleeding is usually similar in members of a single family. Many patients are asymptomatic until the hemostatic system is stressed by surgery or trauma. Treatment is similar to that for hemophilia A. (From Cecil Textbook of Medicine, 19th ed, p1008)
Canadian Medical Guide Definition Hypoprothrombinemias - Absence or reduced levels of prothrombin in the blood.
Canadian Medical Guide Definition von Willebrand Disease - Group of hemorrhagic disorders in which the von Willebrand factor (Factor VIII-related antigen) is either quantitatively or qualitatively abnormal. They are usually inherited as an autosomal dominant trait though rare kindreds are autosomal recessive. Symptoms vary depending on severity and disease type but may include prolonged bleeding time, deficiency of factor VIII, and impaired platelet adhesion.

Coagulation Protein Disorders Medical Definitions and Terms

CANADIAN MEDICAL GUIDE

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