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Bleeding disorders

Coagulopathy

 

Bleeding disorders are a group of conditions in which there is a problem with the body's blood clotting process. These disorders can lead to heavy and prolonged bleeding after an injury. Bleeding can also begin on its own.

Specific bleeding disorders include:

  • Acquired platelet function defects
  • Congenital platelet function defects
  • Congenital protein C or S deficiency
  • Disseminated intravascular coagulation (DIC)
  • Factor II deficiency
  • Factor V deficiency
  • Factor VII deficiency
  • Factor X deficiency
  • Factor XII deficiency
  • Glanzmann disease
  • Hemophilia A
  • Hemophilia B
  • Hemophilia C (Factor XI deficiency)
  • Idiopathic thrombocytopenic purpura (ITP)
  • Von Willebrand's disease (types I, II, and III)

Causes

 

 

Normal blood clotting involves blood components called platelets and as many as 20 different plasma proteins. These are known as blood clotting or coagulation factors. These factors interact with other chemicals to form a substance called fibrin that stops bleeding.

Problems can occur when certain factors are low or missing. Bleeding problems can range from mild to severe.

Some bleeding disorders are present at birth and are passed through families (inherited). Others develop from:

  • Illnesses such as vitamin K deficiency or severe liver disease
  • Treatments such as the use of drugs to stop blood clots (anticoagulants) or the long-term use of antibiotics

Bleeding disorders can also result from a problem with the number or function of the blood cells that promote blood clotting (platelets). These disorders can also be either inherited or develop later (acquired). The side effects of certain drugs often lead to the acquired forms.

 

Symptoms

 

Symptoms may include any of the following:

  • Bleeding into joints or muscles
  • Bruising easily
  • Heavy bleeding
  • Heavy menstrual bleeding
  • Nosebleeds that do not stop easily
  • Excessive bleeding with surgical procedures
  • Umbilical cord bleeding after birth

The type of problems that occur depend on the specific bleeding disorder, and how severe it is.

 

Exams and Tests

 

Tests that may be done include:

  • Complete blood count (CBC)
  • Bleeding time
  • Partial thromboplastin time (PTT)
  • Platelet aggregation test
  • Prothrombin time (PT)

 

Treatment

 

Treatment depends on the type of disorder. It may include:

  • Clotting factor replacement
  • Fresh frozen plasma transfusion
  • Platelet transfusion
  • Other treatments

 

Outlook (Prognosis)

 

Outcome also depends on the disorder. Most primary bleeding disorders can be managed. When the disorders is due to diseases, such as DIC, the outcome will depend on how well the underlying disease can be treated.

 

Possible Complications

 

Complications may include:

  • Bleeding in the brain
  • Severe bleeding (usually from the gastrointestinal tract or injuries)

Other complications can occur, depending on the disorder.

 

When to Contact a Medical Professional

 

Call your health care provider if you notice any unusual or severe bleeding.

 

Prevention

 

Prevention depends on the specific disorder.

 

 

References

Gailani D, Neff AT. Rare coagulation deficiencies. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, Anastasi JI, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 139.

Jobe S, Di Paola J. Congenital and acquired disorders of platelet function. In: Kitchens CS, Kessler CM, Konkle BA, eds. Consultative Hemostasis and Thrombosis. 3rd ed. Philadelphia, PA: Elsevier Saunders: chap 10.

Ragni MV. Hemorrhagic disorders: Coagulation factor deficiencies. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2012:chap 177.

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              Review Date: 1/27/2015

              Reviewed By: Yi-Bin Chen, MD, leukemia/bone marrow transplant program, Massachusetts General Hospital, Boston, MA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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