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Bleeding disorders

Definition

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 or surgery. Bleeding can also begin on its own and may be difficult to stop.

Specific bleeding disorders include:

• Acquired platelet function defects

• Congenital platelet function defects

• Disseminated intravascular coagulation (DIC)

• Prothrombin deficiency

• Factor V deficiency

• Factor VII deficiency

• Factor X deficiency

• Factor XI deficiency (hemophilia C)

• Glanzmann disease

• Hemophilia A

• Hemophilia B

• Idiopathic thrombocytopenic purpura (ITP)

• Von Willebrand disease (types I, II, and III)

Causes

Normal blood clotting involves blood particles, called platelets, and as many as 20 different plasma proteins that layer over the platelets. These proteins 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 platelets are low in number or do not work properly or when certain coagulation factors are low or missing. Bleeding problems can range from mild to severe.

Some bleeding disorders are present at birth and are passed down 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 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 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)

• Partial thromboplastin time (PTT)

• Platelet aggregation test

• Prothrombin time (PT)

• Mixing study, a special PTT test to confirm the factor deficiency

Treatment

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

• Clotting factor replacement

• Fresh frozen plasma transfusion

• Platelet transfusion

• Other treatments

Support Groups

More information and support for people with bleeding disorders and their families can be found at:

• National Hemophilia Foundation -- www.hemophilia.org/bleeding-disorders-a-z/types/other-factor-deficiencies

• National Hemophilia Foundation -- www.victoryforwomen.org/

Outlook (Prognosis)

Outcome also depends on the disorder. Most primary bleeding disorders can be managed. When the disorder 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

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

Prevention

Prevention depends on the specific disorder.

References

Gailani D, Benjamin TF, Wheeler AP. Rare coagulation factor deficiencies. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 135.

Hall JE, Hall ME. Hemostasis and blood coagulation. In: Hall JE, Hall ME, eds. Guyton and Hall Textbook of Medical Physiology. 14th ed. Philadelphia, PA: Elsevier; 2021:chap 37.

Neff AT. Von Willebrand disease and hemorrhagic abnormalities of platelet and vascular function. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 164.

Review Date: 2/2/2023

Reviewed By: Mark Levin, MD, Hematologist and Oncologist, Monsey, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.