Pathogenesis of Hemophilia A – Gpatindia: Pharmacy Jobs, Admissions, Scholarships, Conference,Grants, Exam Alerts

1.] Two of the most common inherited disorders are the sex – (X) – linked disorder – classic hemophilia or hemophilia A (due to the inherited deficiency of factor VIII) and Christmas disease or hemophilia B (due to the inherited deficiency of factor IX).

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HEMOPHILIA – A :-

Hemophilia A is the most common hereditary disorder with serious bleeding.
It is caused due to the reduction in the ability of factor VIII.
This disorder is inherited as a sex – X – linked recessive trait, and therefore, manifests clinically in males, while females are usually the carriers.

PATHOGENESIS :-

Hemophilia A is caused by quantitative reduction of factor – VIII in 90% of the cases, while in 10% of the cases have normal or increased level of factor VIII with reduced activity.
Factor VIII is synthesized in hepatic parenchymal cell and regulates the activation of factor X in intrinsic coagulation pathway.
Normal homeostasis requires 25% factor VIII activity.
Occasional patients with 25% factor VIII level may develop bleeding, most symptomatic hemophilia patient have factor VIII level below 5%.

TREATMENT :-

It involves infusion of factor VIII, currently derived from human plasma.
Symptomatic patients with bleeding episode are treated with factor VIII replacement therapy.

HEMOPHILIA B :-

Hemophilia B is also known as Christmas disease named after the first patient diagnosed with this disorder.
It is caused due to the inherited deficiency of factor IX.
It is also an inherited X – linked recessive trait and may occur asymptotically or with associated hemorrhage.
As with hemophilia A the partial thromboplastin time is prolonged, and bleeding time is normal.
Diagnosis of this disease id possible only by the assay of factor level.

TREATMENT :-

Therapy in asymptomatic hemophilia B consist of infusion of either fresh frozen plasma or a plasma enriched with factor IX.
The replacement therapy in factor IX deficiency may activate the coagulation system and causes thrombosis and embolism.

MULTIPLE CHOICE QUESTION :-

1.] Hemophilia is caused by ?

a. Bacteria

b.Virus

c. Genetic mutation

d. Cause unknown

2.] This is a complication of hemophilia ?

a. Bleeding in the head

b. Joint swelling

c. Death

d. All of the above

3.] Hemophilia is a result of lack of ?

a. STH

b. ADH

c. AHF

d. ACTH

4.] Hemophilia is ?

a. X – linked

b. Y – linked

c. Z – linked

d. Autosomal

5.] This lack in patient with hemophilic – A ?

a. X – chromosome

b. Calcium

c. Antihemophilic factor

d. Plasma thromboplastin

6.] This is ineffective against antibiotics ?

a. Bacterial infected gonorrhea

b. Bacterial infected wound

c. Hemophilia

d. Bacterial infected throat

7.] Only men can get hemophilia ?

a. True

b. False

8.] What is the main symptom of hemophilia ?

a.Bleeding

b. Headache

c. Fatigue

d. Muscle pain

9.] Another name for hemophilia is Von Willebrand disease ?

a. True

b. False

10.] What is the treatment for hemophilia ?

a. Surgery

b. Gene therapy

c. Diet

d. Replacing the clotting factor

SOLUTION :-

1.] (c) Genetic mutation

2.] (d)

3.] (c) AHF

4.] (a) X – Linked

5.] (c) Antihemophilic factor

6.] (c) Hemophilia

7.] (b)

8.] (a) Bleeding

9.] (b) False

10.] (d) Replacing the clotting factor

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REFERENCES :-

1.] Textbook of Pathology By Harsh Mohan; 7th edition; Page no.313 – 314.

2.] Robbin’s Basic Pathology; 5th edition; Page no.622 – 623.

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