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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-a8/12/2019 Tambahan Utk Case Report
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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
40 U/kg factor IX concentrate
; may need treatment every
23 days until resolved
Mouth, deciduous
tooth, or toothextraction
20 U/kg factor VIII concentrate;
antifibrinolytic therapy; removeloose deciduous tooth
40 U/kg factor IX concentrate
; 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%.
30 U/kg factor IX concentrate
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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