Hemophilia

Answer 1

Hemophilia is defines as a group of diseases characterized by an abnormal tendency to bleed profusely from the slightest wound and/or hemorrhage. This inherited bleeding disorder is causes by a deficiency of particular blood proteins, called factors. Due to an insufficiency or absence of certain blood proteins, hemophiliacs blood clotting process is extremely slow. The inability to stop bleeding rapidly results in life-threatening incidents. hemophilia is diagnoses by blood clotting tests that reveal factor VII activity abnormally low. Not only have the complications associated with the disease itself proved to be life-threatening, but it's treatment methods as well.

Answer 2

Definition

Hemophilia refers to a group of bleeding disorders in which it takes a long time for the blood to clot.

Related topics:

• Hemophilia A
• Hemophilia B
• von Willebrand disease

Causes, incidence, and risk factors

Blood clotting factors are substances in the blood that help form a clot. When one or more of these clotting factors are missing, there is a higher chance of bleeding.

In most cases, the disorder is passed down through families (inherited). It most often affects males.

Symptoms

The main symptom of hemophilia is bleeding. Mild cases may go unnoticed until later in life, when they occur during surgery or after trauma.

In more severe cases, serious bleeding may occur without any cause. Internal bleeding may occur anywhere. Bleeding into joints is common.

Signs and tests

Most often, hemophilia is diagnosed after a person has an abnormal bleeding episode or when there is a known family history of the condition.

TreatmentStandard treatment involves replacing the missing clotting factor. Support Groups

See: Hemophilia - Resources

Expectations (prognosis)

Most people with hemophilia are able to lead relatively normal lives. However, some patients have significant bleeding events, most commonly chronic bleeding into the joint spaces.

A small percentage of people with hemophilia may die from severe bleeding.

References

Kumar V, Abbas AK, Fausto N. Robbins and Cotran Pathologic Basis of Disease. 7th ed. St. Louis, Mo: WB Saunders; 2005:655-656.

Kessler C. Hemorrhagic disorders: Coagulation factor deficiencies. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 180.

Answer 3

Definition

Hemophilia refers to a group of bleeding disorders in which it takes a long time for the blood to clot.

Related topics:

• Hemophilia A
• Hemophilia B
• Von Willebrand disease

Causes, incidence, and risk factors

When you bleed, the body launches a series of reactions that help the blood clot. This is called the coagulation cascade. The process involves special proteins called coagulation factors. When one or more of these clotting factors are missing, there is usually a higher chance of bleeding.

Hemophilia is caused by a lack of enough factor VIII or IX. In most cases, hemophilia is passed down through families (inherited). It most often affects males.

Symptoms

The main symptom of hemophilia is bleeding. Mild cases may go unnoticed until later in life, when they occur during surgery or after trauma.

In more severe cases, serious bleeding may occur without any cause. Internal bleeding may occur anywhere. Bleeding into joints is common.

Signs and tests

Most often, hemophilia is diagnosed after a person has an abnormal bleeding episode or when there is a known family history of the condition.

Treatment

Standard treatment involves replacing the missing clotting factor through a vein (intravenous infusions).

Diagnosing a bleeding disorder is important so that the doctor can take extra care if you need surgery, and can test or warn other family members who might be affected.

Support Groups

See: Hemophilia - Resources

Expectations (prognosis)

Many people with hemophilia are able to lead pretty normal lives. However, some patients have major bleeding events, most commonly bleeding into the joint spaces.

A small percentage of people with hemophilia may die from severe bleeding.

References

Kessler C. Hemorrhagic disorders: Coagulation factor deficiencies. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 180.

Ragni MV, Kessler CM, Lozier JN. Clinical aspects and therapy for hemophilia. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al, eds. Hoffman Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Churchill Livingstone Elsevier;2008:chap 2005.

Reviewed By

Review Date: 02/28/2011

David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.