Hemophilia

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Hemophilia is a rare inherited bleeding disorder caused by factor deficiency of either clotting factor VIII or factor IX characterized by  prolonged bleeding. Hemophilia is a lifelong disease. Hemophilia is divided into hemophilia A, hemophilia B and hemophilia C.

What causes the Hemophilia?

Hemophilia is a caused by a deficiency of the clotting factors. Type of hemophilia depends on which clotting factor is deficient.

1.  Hemophilia A: Caused by deficiency of clotting factor VIII.
2. Hemophilia B: It is also called Christmas disease caused by deficiency of clotting factor IX.
3.  Hemophilia C: Caused by deficiency of clotting factor XI.

What are the signs symptoms of Hemophilia?

Sings and symptoms are not depending upon the type of hemophilia but depending on severity of factor deficiency and the location of the disease.

Symptoms include:

1. Large or deep
2. Joint pain or joint swelling
3. Unexplained blood in urine or in stool
4. Nose bleeding
5. Unexplained bleeding or bruising
6. Excessive bleeding from biting
7. Excessive bleeding during tooth extraction or during surgery

Some people present with the emergency sings and symptoms like

• Sudden pain, swelling of large joints such as knees, elbows, hips and shoulder.
• Bleeding from an injury
• Headache
• Vomiting
• Neck pain or Double vision

How to diagnosed hemophilia?

A family history of bleeding disorder aids in diagnosing hemophilia. But certain test help to diagnose the disease.

Deficiency of a clotting factor is the confirmative test for Hemophilia.

Is there a cure of hemophilia?

There is no cure of hemophilia but some treatment available that can effectively reduce the symptoms and complication of the disease. Treatment of hemophilia depends on the severity of the condition:

In mild hemophilia patients is managed with hormone desmopressin (DDAVP). Moderate and severe hemophilia patients are managed with an infusion of clotting factor, fresh plasma and cryoprecipitate.

References:

• Sabiston textbook of surgery 18th edition
• The Washington manual of surgery, 5th edition.
• emedicine.medscape.com
• Harrison’s Principles of Internal Medicine, 17th edition.
• Davidson’s Principles and Practice of Medicine, 20th Edition

Filed Under: Hematology • Medicine • Surgery

Tags: bleeding disorder, featured, Hemophilia, hemophilia A, hemophilia B, hemophilia C