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Thomson A., 2008.Hemophilia A. Available from:

http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=hemo-a. [Accesed 21

March 2010].
http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=hemo-ahttp://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=hemo-ahttp://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=hemo-a


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Coagulation cascade and laboratory assessment of clotting factor deficiency by

activated partial prothrombin time (aPTT), prothrombin time (PT), and thrombin time

(TT)

General guideline for factor replacement for Treatment of bleeding in Hemophilia

Type of Hemorrhage Hemophilia A Hemophilia B

Hemarthrosis * 2040 U/kg factor VIII

concentrate ;15 U/kg if treated

early. Repeat the dose daily untiljoint function is normal or back to

baseline. Consider additional

treatment every other day for 7

10 days. Consider prophylaxis.

40 U/kg factor IX concentrate

; 30 U/kg if treated early.

Repeat the dose daily untiljoint function is normal or

back to baseline. Consider

additional treatment every

other day for 710 days.

Consider prophylaxis.

Muscle or significant

subcutaneous

hematoma

20 U/kg factor VIII concentrate;

may need every-other-day

treatment until resolved


; may need treatment every

23 days until resolved

Mouth, deciduous

tooth, or toothextraction

20 U/kg factor VIII concentrate;

antifibrinolytic therapy; removeloose deciduous tooth


; antifibrinolytic therapy ;remove loose deciduous tooth


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Type of Hemorrhage Hemophilia A Hemophilia B

Epistaxis Apply pressure for 1520 min;

pack with petrolatum gauze;

antifibrinolytic therapy; 20 U/kg

factor VIII concentrate if abovefails

Apply pressure for 1520

min; pack with petrolatum

gauze; antifibrinolytic

therapy; 30 U/kg factor IXconcentrate if above fails

Major surgery, life-

threatening

hemorrhage (e.g.,

central nervous

system,

gastrointestinal,

airway)

5075 U/kg factor VIII

concentrate, then initiate

continuous infusion of 24

U/kg/hr to maintain factor VIII

>100 U/dL for 24 hr, then give 2

3 U/kg/hr continuously for 57

days to maintain the level > 50

U/dL and an additional 57 days

at a level > 30 U/dL

120 U/kg factor IX

concentrate , then 5060

U/kg every 1224 hr to

maintain factor IX > 40 U/dL

for 57 days, and then > 30

U/dL for 5 days

Iliopsoas hemorrhage 50 U/kg factor VIII concentrate,

then 25 U/kg every 12 hr until

asymptomatic, then 20 U/kg

every other day for total 1014

days

120 U/kg factor IX

concentrate ; then 5060

U/kg every 1224 hr to

maintain factor X > 40 U/dL

until asymptomatic, then 40

50 U every other day for total

1014 days I

Hematuria Bed rest; maintenance fluids;

if not controlled in 12 days, 20

U/kg factor VIII concentrate; if

not controlled, give prednisone

(unless HIV-infected)

Bed rest: maintenance

fluids; if not controlled in 12

days, 40 units/kg factor IX

concentrate ; if not

controlled, give prednisone

(unless HIV-infected)

Prophylaxis 20 U/kg factor VIII concentrate

every other day to achieve trough

level 1%.


every 23 days to achieve

trough level 1%

Adapted from Montgomery RR, Gill JC, Scott JP: Hemophilia and von Willebrand

disease. In Nathan DG, Orkin SH (editors): Nathan and Oski's Hematology of Infancy

and Childhood, 5th ed. Philadelphia, WB Saunders, 1998.*

For hip hemarthrosis, orthopedic evaluation for possible aspiration is advisable to preventavascular necrosis of the femoral head.

For mild or moderate hemophilia, desmopressin, 0.3g/kg, should be used instead of factor

VIII concentrate, if patient is known to respond with a hemostatic level of factor VIII; ifrepeated doses are given, monitor factor VIII levels for tachyphylaxis.

Stated doses apply for recombinant factor IX concentrate; for plasma- derived factor IXconcentrate, use 70% of stated dose.

Do not give antifibrinolytic therapy until 46 hr after a dose of prothrombin complex

concentrate.

Repeat radiologic assessment should be performed before discontinuation of therapy,

If repeated doses of factor IX concentrate are required, use highly purified, specific factor IX


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concentrate.

Patient Diagnosis and treatment

Coagulation disorders are founded in the patient with any of the following like

gum bleeding and subconjuctival bleeding on the left eye in 2006. The patient also had

mild malnutrition.

From the laboratorium, the patient had a low hemoglobin and the APTT was

prolonged. The factor VIII clotting activity was lower than a normal (1.24 %) and

classified as a moderately severe hemophilia A. the test had been done on 23th January

2006. But the laboratory finding on March 2010 showed the low F IX (43.9 %).

The patient had been treated with antihemophilic factor human derived (koate)

and recombinant FVIII concentrate (koagenate) and transfusion of wash PRC. The patient

also had a diet therapy with 1720 kkal and 62 gram of protein.

Complication and prognosis of this patient

This patient can had an intracranial hemmorage, cronic hemarthrosis and infection. But

with continued appropriate education and treatment, this patient can live full and

productive lives.

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