In medicine (hematology), bleeding diathesis is an unusual susceptibility to bleed (hemorrhage) mostly due to hypocoagulability (a condition of irregular and slow blood clotting), in turn caused by a coagulopathy (a defect in the system of coagulation). Therefore, this may result in the reduction of platelets being produced and leads to excessive bleeding. Several types of coagulopathy are distinguished, ranging from mild to lethal. Coagulopathy can be caused by thinning of the skin (Cushing's syndrome), such that the skin is weakened and is bruised easily and frequently without any trauma or injury to the body. Also, coagulopathy can be contributed by impaired wound healing or impaired clot formation.
Signs and symptoms
Symptom DisordersPetechiae (red spots) Wiskott–Aldrich syndrome, where they may resemble a few bruisesWiskott–Aldrich Syndrome The International Patient Organisation for Primary Immunodeficiencies (IPOPI). Acute leukemiaDisorders of thrombosis and hemostasis: clinical and laboratory practice. Page Rodger L. Bick. Edition 3, illustrated. , . 446 pages Chronic leukemia Vitamin K deficiencyVitamin K Deficiency eMedicine. Author: Pankaj Patel, MD. Coauthor(s): Mageda Mikhail, MD, Assistant Professor. Updated: Dec 18, 2008 Purpura and ecchymoses Acute leukemia Chronic leukemia Vitamin K deficiency Blood in stool Wiskott–Aldrich syndrome, especially in infancy Acute leukemia Bleeding gingiva (gums) Wiskott–Aldrich syndrome Acute leukemia Chronic leukemia Prolonged nose bleeds Wiskott–Aldrich syndrome
Complications Following are some complications of coagulopathies, some of them caused by their treatments:
Complication Disorders Soft tissue bleeding, e.g. deep-muscle bleeding, leading to swelling, numbness or pain of a limb. HemophiliaHemophilia Complications By Mayo Clinic staff. May 16, 2009 Von Willebrand disease Joint damage, potentially with severe pain and even destruction of the joint and development of arthritis Hemophilia Von Willebrand disease Retinal bleeding Acute leukemia Transfusion transmitted infection, from blood transfusions that are given as treatment. Hemophilia Adverse reactions to clotting factor treatment. Hemophilia Anemia Von Willebrand diseaseVon Willebrand disease --> Complications By Mayo Clinic staff. Feb. 7, 2009 Exsanguination (bleeding to death) Von Willebrand disease Acute leukemia Vitamin K deficiency Cerebral hemorrhage Wiskott–Aldrich syndrome
Causes While there are several possible causes, they generally result in excessive bleeding and a lack of clotting.
Acquired Acquired causes of coagulopathy include anticoagulation with warfarin, liver failure, vitamin K deficiency and disseminated intravascular coagulation. Additionally, the hemotoxic venom from certain species of snakes can cause this condition, for example Bothrops, rattlesnakes and other species of viper. Viral hemorrhagic fevers include dengue hemorrhagic fever and dengue shock syndrome. Leukemia may also cause coagulopathy. Furthermore, cystic fibrosis has been known to cause bleeding diathesis, especially in undiagnosed infants, due to malabsorption of fat soluble vitamins like vitamin K.
Autoimmune causes of acquired coagulation disorders There are autoimmune causes of coagulation disorders. They include acquired antibodies to coagulation factors, termed inhibitors of coagulation. The main inhibitor is directed against clotting factor VIII. Another example is antiphospholipid syndrome, an autoimmune, hypercoagulable state.
Causes other than coagulation Bleeding diathesis may also be caused by impaired wound healing (as in scurvy), or by thinning of the skin, such as in Cushing's syndrome.
Genetic Some people lack genes that typically produce the protein coagulation factors that allow normal clotting. Various types of hemophilia and von Willebrand disease are the major genetic disorders associated with coagulopathy. Rare examples are Bernard–Soulier syndrome, Wiskott–Aldrich syndrome and Glanzmann's thrombasthenia. Gene therapy treatments may be a solution as they involve in the insertion of normal genes to replace defective genes causing for the genetic disorder. Gene therapy is a source of active research that hold promise for the future.
Diagnosis
Comparing coagulation tests + Laboratory findings in various platelet and coagulation disorders Condition Prothrombin time Partial thromboplastin time Bleeding time Platelet count Vitamin K deficiency or warfarin Prolonged Normal or mildly prolonged Unaffected Unaffected Disseminated intravascular coagulation Prolonged Prolonged Prolonged Decreased Von Willebrand disease Unaffected Prolonged or unaffected Prolonged Unaffected Hemophilia Unaffected Prolonged Unaffected Unaffected Aspirin Unaffected Unaffected Prolonged Unaffected Thrombocytopenia Unaffected Unaffected Prolonged Decreased Liver failure, early Prolonged Unaffected Unaffected Unaffected Liver failure, end-stage Prolonged Prolonged Prolonged Decreased Uremia Unaffected Unaffected Prolonged Unaffected Congenital afibrinogenemia Prolonged Prolonged Prolonged Unaffected Factor V deficiency Prolonged Prolonged Unaffected Unaffected Factor X deficiency as seen in amyloid purpura Prolonged Prolonged Unaffected Unaffected Glanzmann's thrombasthenia Unaffected Unaffected Prolonged Unaffected Bernard–Soulier syndrome Unaffected Unaffected Prolonged Decreased or unaffected Factor XII deficiency Unaffected Prolonged Unaffected Unaffected C1INH deficiency Unaffected Shortened Unaffected Unaffected
Treatments Consult a hematologist and have regular blood check ups. Have an early diagnostic test for any blood disorders or blood diseases including hemophilia, hemorrhage, and sickle-cell anemia. Prothrombin time and partial thromboplastin time blood tests are useful to investigate the reason behind the excessive bleeding. The PT evaluates coagulation factors I, II, V, VII and X, while the PTT evaluates coagulation factors I, II, V, VIII, IX, X, XI and XII. The analysis of both tests thus helps to diagnose certain disorders.
Blood transfusion involves the transfer of plasma containing all the necessary coagulating factors (fibrinogen, prothrombin, thromboplastin) to help restore them and to improve the immune defense of the patient after excessive blood loss. Blood transfusion also caused the transfer of platelets that can work along with coagulating factors for blood clotting to commence.
Different drugs can be prescribed depending on the type of disease. Vitamins (K, P and C) are essential in case of obstruction to walls of blood vessels. Also, vitamin K is required for the production of blood clotting factors, hence the injection of vitamin K (phytomenadione) is recommended to boost blood clotting.
References
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