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Medicine

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Hematology

Question
3 out of 9
 

In a patient of Hemophilia to be taken for dental extraction true is all, except (AIIMS Nov 10)



A All patients should be screened for HIV

B Extraction should be done under general anaesthesia and skilled anaesthetic care

C Factor VIII or cryoprecipitate can be needed

D Dose of Lignocaine required for anaesthesia is same as that for normal individuals

Ans. B

Extraction should be done under general anaesthesia and skilled anaesthetic care

Management

1. Bleeding episodes are treated by factor VIII concentrate infusion (from donor plasma).

2. IV desmopressin 0.3 mg/kg, can be given to raise factor VIII level to three to five fold.

3. Cryoprecipitate is rich in factor VIII concentrate

4. Antifibrinolytic drugs - E amino caproic acid (EACA), Tranexamic acid

Haemophilia b (christmas disease)

1. Haemophilia B is a blood clotting disorder caused by a mutation of the Factor IX gene, leading to a deficiency of Factor IX.

2. It is x-linked recessive disorder.

3. It is also called Christmas daisease,

4. Treatment (bleeding prophylaxis) is by intravenous infusion of factor IX. Factor IX has a longer half life than factor VIII (Deficient in Haemophilia A) and as such factor IX can be transfused less frequently.

Haemophilia A (Classical hemophilia)

Haemophilia B (Christmas disease)

Defect

Pathway of coagulation affected

Whole blood clotting time

PTT

PT

BT

Platelet count

Deficiency of coagulant subunit of factor i.e. (VIII C)

Factor VIII = VIII c + VWF

Intrinsic pathway

N

N

N

Deficiency of factor IX.

Intrinsic pathway

N

N

N

Hematology Flashcard List

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