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 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:
- Airway obstruction caused by bleeding into the neck and pharynx.
- Intracranial bleeding leading to neurological impairment.
- Intestinal obstruction due to GI bleeding.
- Compartment syndrome due to compression of nerves by bleeding into deep tissues.
- Degenerative joint disease, osteoporosis and muscle atrophy due to repeated hemorrhage.
- Chronic hepatitis and cirrhosis due to contaminated cryoprecipitate.
- HIV/AIDS due to transfusion related infections.
- 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.
Maria Khatun Mona is a Founder and Editor of Nursing Exercise Blog. She is a Nursing and Midwifery Expert. Currently she is working as a Registered Nurse at Evercare Hospital, Dhaka, Bangladesh. She has great passion in writing different articles on Nursing and Midwifery. Mail her at “[email protected]”
