Bleeding disorders

 

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 that stops bleeding called fibrin.

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 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 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 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.

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 depends on the type of disorder. It may include:

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

Find out more about bleeding disorders through these groups:

• American Society of Hematology: Blood Disorders -- www.hematology.org/Patients/Blood-Disorders.aspx
• National Hemophilia Foundation: Other Factor Deficiencies -- www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Other-Factor-Deficiencies
• National Hemophilia Foundation: Victory for Women with Blood Disorders -- www.hemophilia.org/Community-Resources/Women-with-Bleeding-Disorders/Victory-for-Women-with-Blood-Disorders

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.

Complications may include:

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

Other complications can occur, depending on the disorder.

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

Prevention depends on the specific disorder.

Gailani D, Neff AT. Rare coagulation deficiencies. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Elsevier Saunders; 2013:chap 139.

Hall JE. Hemostasis and blood coagulation. In: Hall JE, ed. Guyton and Hall Textbook of Medical Physiology. 13th ed. Philadelphia, PA: Elsevier; 2016:chap 37.

Nichols WL. Von Willebrand disease and hemorrhagic abnormalities of platelet and vascular function. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 173.

Ragni MV. Hemorrhagic disorders: coagulation factor deficiencies. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 174.