Coagulopathy (bleeding disorder)

CoagulopathyCoagulopathy (bleeding disorder) – an impairment of the blood coagulation ability.

 

Description

The hemostatic system includes the platelets, coagulation factors, and the endothelial cells lining the blood vessels. Coagulation is the process required to stop the bleeding by the formation of the blood clot with a help of platelets (thrombocytes) and fibrin (protein).

13 clotting factors are necessary fr the effective coagulation. If any of them is defective or deficient, the blood clotting is impaired; as a result occurs a bleeding disorder.

Coagulopathy is a condition characterised by the prolonged or excessive bleeding (so called bleeding diathesis), which may occur spontaneously or due to an injury (even tiny), any medical or dental procedure.

 

Causes

Coagulopathy may be the result of certain genes that an individual has inherited or caused by conditions or factors that develop within a person’s lifetime (acquired). Bleeding disorder may occur when there is not enough platelets (thrombocytopenia) or their function is impaired, or when a person has low serum levels of coagulation factors.Thrombocytes are not only involved in the coagulation but also assure the lasting of the blood vessels, thereafter the platelet dysfunction leads to the vessel fragility.

 

Risk factors

Some of the bleeding disorders are hereditary. Having a relative with a coagulopathy puts a person into the risk of having a bleeding disorder. The liver is the main body manufacture where all the essential substances are produced. Liver diseases may cause bleeding disorders due to its inability to produce proteins involved in coagulation. Vitamin K is needed for the production of coagulation factors and, therefore, its deficiency results in the coagulation factors X, IX, VII, II deficiency.

Prolonged intake of anticoagulants (Warfarin) if not regularly estimated, may cause bleeding disorder. Long-term administration of NSAIDs (aspirin, ibuprofen) may also affect hemostasis.

A lot of life-threatening conditions (sepsis, intoxication, severe trauma, etc.) may cause disseminated intravascular coagulation (DIC), characterized by the impairment of coagulation.

See also: Pseudocholinesterase deficiency

Types

  • Haemophilia – haemophilia A, a condition, which occurs due to the clotting factor VIII deficiency, and haemophilia B – when the deficiency of the clotting factor IX is present. The conditions are inherited with a X-linked inheritance pattern and therefore extremely rare in females and common in males;
  • Haemophilia C is a factor XI deficiency with autosomal recessive inheritance pattern;
  • Von Willebrand’s disease (types I, II, and III) is caused by the deficiency of von Willebrand factor or its low quality; this factor is necessary for the platelet adhesion;
  • Acquired platelet function defects are seen in numerous disorders,  including idiopathic thrombocytopenic purpura (ITP), chronic myelogenous leukemia, multiple myeloma, primary myelofibrosis, polycythemia vera, primary thrombocythemia, thrombotic thrombocytopenic purpura, kidney (renal) failure, they may occur due to some medicine intake such as aspirin, ibuprofen, other anti-inflammatory drugs, penicillin, phenothiazines, and prednisone (after long-term use);
  • Congenital platelet function defects include

o Bernard-Soulier syndrome – a rare disorder with unusually large platelets, low platelet count (thrombocytopenia) and prolonged bleeding time;

o Glanzmann thrombasthenia – a rare condition caused by the lack of glycoprotein IIb/IIIa (GpIIb/IIIa) needed for platelets to clump together;

o Platelet storage pool disorder (platelet secretion disorder) occurs when the platelets are not able to store the substances necessary for the coagulation or the platelets are not able to release these substances properly and particularly when the lack of granular non-metabolic ADP is detected;

  • Disseminated intravascular coagulation (DIC) – a disorder characterized by the widespread activation of the clotting cascade and, as a result, occur a lot of blood clots throughout the body. Later, as the coagulation process uses clotting factors and platelets, normal clotting is disrupted and develops severe bleeding in various sites;
  • Hypoprothrombinemia (Factor II deficiency);
  • Factor V deficiency;
  • Factor VII deficiency may be acquired in case of vitamin K deficiency or inherited;
  • Factor X deficiency;
  • Factor XII deficiency (Hageman factor deficiency) may be inherited or acquired;

 

Symptoms

Symptoms vary depending on the type of disorder and its severity.

Symptoms may include any of the following:

  • Bleeding into joints – hemarthroses (commonly ankles in children and knees and elbows in adults) or muscles (typically quadriceps and iliolpsoas);
  • Mouth bleeding of gastrointestinal bleeding;
  • Bruising easily or with no visible reason;
  • Heavy bleeding;
  • Heavy menstrual bleeding (lasting longer than 7 days or excessive bleeding during the menstrual period (more than 80 ml per cycle);
  • Frequent nosebleeds (epistaxis);
  • Excessive bleeding from injuries or cuts;
  • Excessive bleeding during the surgeries;
  • Umbilical cord bleeding after birth;

Internal bleeding may be life-threatening.

 

Diagnosis

Tests that may be done include:

  • Complete blood count (CBC);
  • Bleeding time;
  • Partial thromboplastin time (PTT), platelet aggregation test,  prothrombin time (PT);
  • Concentration of the clotting factors in the blood;

 

Treatment

When the bleeding occurs in a person with coagulopathy it’s extremely hard to stop it. Then they require transfusion of a combination of red cells with blood plasma, prothrombin complex concentrate, factor XIII, and fibrinogen or fibrinogen with tranexamic acid to stop the bleeding.

Treatment of bleeding disorders varies according to a type of disorder and may include:

  • Factor concentrates – individual concentrates for factors I, VII, VIII, XI, and XIII, recombinant factor VIII and recombinant factor VIIa are available.;
  • Prothrombin complex concentrate (PCC) includes factors II, VII, IX, and X;
  • Fresh frozen plasma (FFP) contains all the factors required for coagulation;
  • Cryoprecipitate contains factor VIII, fibrinogen (factor I) along with some other factors;
  • Desmopressin is a synthetic hormone that elevates the concentration of factor VIII in the serum;
  • Antifibrinolytic drugs (tranexamic acid and aminocaproic acid);
  • Fibrin glue;
  • Platelet transfusions;
  • Vitamin K (phytonadione);
  • Hormonal Contraceptives or Intra-uterine devices (IUDs) to control menstrual bleeding;