Symptoms, Types and Complications of Hemophilia Disease

Definition of Hemophilia Disease:

Hemophilia is an inherited bleeding disorder due to deficiency of plasma coagulation factors. It is primarily found in males but transmitted by female carriers.

Hemophilia Disease
Fig: Hemophilia Disease

Hemophilia is a rare disorder in which your blood doesn’t clot normally because it lacks sufficient blood- clotting proteins (clotting factors).

Classification of Hemophilia Disease:

Based on the deficient factors of coagulation:

1. Hemophilia-A (classical hemophilia):

It occurs due to deficiency of plasma factor VUI, the antihemophilic factor (AHF). It accounts for 80 to 85 percent of all hemophilics. Hemophilia- A can be classified based upon the factor-VIII level in plasma. They are as follows:

a. Severe hemophilia-A:

In this condition, the factor level is found less than l percent of normal value. Patients have tendency of spontaneous bleeding and severe bleeding.

b. Moderate hemophilla-A:

Factor level remains between 1 to 5 percent of normal. Patients have no spontaneous bleeding and may not have severe bleeding until any trauma occurs.

c. Mild hemophilia-A:

Factor level is in between6 to 30 percent of normal. Patients usually lead normal lives.

2. Hemophilia-B (Christmas disease):

It results from deficiency of plasma factor DX, the plasma thromboplastin component (PTC). It accounts for about 15 to 20 percent of cases.

3. Hemophilia-C:

It results from deficiency of factor XI, plasma thromboplastin antecedent (PTA). It accounts for few cases only.

Clinical Features of Hemophilia:

Sign and Symptoms of Hemophilia Disease:

It includes the following:

  • Unusual bleeding after vaccinations Unexplained and excessive bleeding from cuts or injuries, or after surgery or dental work.
  • Many large or deep bruises.
  • Pain, swelling or tightness in your joints.
  • Blood in your urine or stool.
  • Nosebleeds without a known cause.
  • In infants, unexplained irritability.

Complications of Hemophilia:

Hemophilia may cause numbers of complications. They include:

  1. Airway obstruction caused by bleeding into the neck and pharynx.
  2. Intracranial bleeding leading to neurological impairment.
  3. Intestinal obstruction due to GI bleeding.
  4. Compartment syndrome due to compression of nerves by bleeding into deep tissues.
  5. Degenerative joint disease, osteoporosis and muscle atrophy due to repeated hemorrhage.
  6. Chronic hepatitis and cirrhosis due to contaminated cryoprecipitate.
  7. HIV/AIDS due to transfusion related infections.
  8. Physical, psychological and social handicaps.

More questions related to this article:

  • Define hemophilia.
  • What is hemophilia?
  • What do you mean by hemophilia?
  • Classify hemophilia.
  • Write down the types of hemophilia.
  • Enumerate the clinical features of hemophilia.
  • What are the sign and symptoms of hemophilia?
  • State the complications of hemophilia.

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