Question

A 36 year old actress in apparently good health was admitted to the hospital for cosmetic surgery. No abnormalities were noted on her history or physical exam. Family history was unremarkable. Her routine admission tests for surgery were as follows:

PT: 11.3 sec.

PTT: 58.0 sec. (corrected by adsorbed plasma and aged serum

BT: 3 min.

TT: 11 sec.

Plt count: 198,000/uL

1. Possible deficiency?

2. Treatment? yes or no

3. If so, what?

Answer 1

1.Her laboratory results sugest that she is sufering from Hemophilia

Laboratory results in Hemophilia

Test	Results in hemophilia	Patient values	Inference
1.Prothrombin Time	Normal . No involvement of extrinsic system	11.3 sec	Normal (11-16 sec)
2.Thrombin Time	Normal. No impairement of thrombin-fibrinogen reation	11 sec	Normal (11-23 sec)
3. Platelet Count	Normal. Adequate platelet function	198,000/uL	Normal (150,000-300,000/uL)
4. Partial Thromboplastin Time	Prolonged. Because of deficiency in intrinsic clotting system factor	58.0 sec	prolonged (25-35 sec)
5. Bleeding time	Normal in Hemophilia A &B because platelets not affected	3 mts	Normal (2-7 mts)

Hemophilia is an X-linked genetic disorder caused by defective or deficient coagulation factor

The two major types of hemophilia are,

Hemophilia A (classic hemophilia, Factor VIII deficiency)

Hemophilia B ( Christmas Disease, Factor IX deficiency)

Treatment

Replacement of deficient clotting factors is a primary means of supporting a patient with hemophilia

In addition to treating acute crisis replacement therapy should be given prior to surgery and dental care as prophylatic measure.

Drug Therapy- Replacement therapy for Hemophilia

Factor VIII	Factor IX	For Patients who have inhibitors
Advate Alphanate Helixate FS Hemophil M Humate P Monoclate P	AlphaNine SD Beublin VH Benefix Mononine	NovoSeven Factor Autoplex T Factor VIII inhibitor bypassing activity

Fresh Frozen Plasma, once commonly used for replacement therapy is rarely used today

Surgery and traumatic injuries require more prolonged therapy