Hemophilia
Hemophilia
Hemophilia
k25
DIVISI HEMATOLOGI-ONKOLOGI
DEPARTEMENT OF CHILD HEALTH
H.ADAM MALIK HOSPITAL
MEDAN
Introduction
X
HEMOPHILIA. A
Is a bleeding disorder that results from a
deficiency in coagulation factor VIII (FVIII).
HEMOPHILIA B
Is a bleeding disorder that result from a
deficiency in coagulation factor IX (F IX)
FAMILY HISTORY
FATHER
MOTHER
: HEMOPHILIA
: NORMAL
FATHER : NORMAL
MOTHER
: CARIER
FATHER : HEMOPHILIA
MOTHER
: KARIER
PREVALENCE OF HEMOPHILIA
Indonesia
Population
Expected Prevalence
Known cases
Characterised
209 million
13,000*
850 (6.5%)
234
GNI
Prevalence
FVIII p.c.
35,400
51
3.4
2,830
37
0.93
710
0.008
CLINICAL MANIFESTATION :
Bleeding
on muscle
and joint
Severe pain
recurrent
Continous bleeding
Life threatining
Laboratory finding
Reduce
Treatment
guideline therapy for hemorrhage
in hemophilia (1)
Site bleed
Hemostatic factor
level
Factor dosing
hemophilia A
Comment
Joint
40-80%
20-40 u/kgBW
qd as needed
-rest/immobilization
-physical/rehabilitation
following bleed
muscle
40-80%
20-40 u/kgBW
Qd as needed
Oral mucosa
25 u/kgBW
gastrointestinal
Antifibrinolytic therapy
maybe usefull
Treatment
guideline therapy for hemorrhage
in hemophilia(2)
Site bleed
Hemostatic factor
level
Factor dosing
hemophilia A
Comment
Epistaxis
Initially 80-100%
then 30% until
healing occurs
Pressure/pack/cautery
usefull for severe or
recurrent bleed
Genitourinary
Central nervous
system
50 u/kg then
25u/kg q12 hours
or continous
infusion
Anticonvulsant frequently
used preventively
Trauma or surgery
Factor XI Deficiency
( Hemophilia C )
An
autosomal deficiency
Mild and moderate bleeding symptoms
In Israel 1-3/1000 are homozygous
The bleeding not correlated with the amount of
Factor XI
Severe deficiency minimal or no symptoms
Treatment
There
is no concentrate factor XI
Fresh Frozen Plasma (FFP)
Minor surgery local pressure
Plasma infusion 1U/kg increase
plasma
concentration by 2 U/dl
The infusion of 10 -15ml/kg plasma
sufficient to control moderate
hemorrhage
bleeding disorder
1 2% of general population
Inherited autosomal
Women > men
Classified : type 1, 2 and 3
Clinical manifestations
Mucocutaneous
-
hemorrhage
Excessive bruising, epistaxis, menorrhagia
Post op hemorrhage
Laboratory findings
Bleeding time
PTT
Platelet count was normal
Treatment
Increasing
Factor
VIII
Desmopressin (DDAVP)
Plasma derived VWF : 1 U/kg 1.5
U/dl
Purified VWF concentrate future
- presurgical management or
prophylaxis
Specific hemorrhage
Joint hemorrhage
specific hemorrhage
Muscle hemorrhage
Should be monitored
neurovascular compromise
specific hemorrhage
Iliopsoas hemorrhage
Hospitalize for
observation,treat anemia
CT scan for confirm the
diagnosis.
Limit activity until pain
resolves,physical therapy
specific hemorrhage
specific hemorrhage
specific hemorrhage
specific hemorrhage
Evaluation by
ophtalmologist and
hematologist is required
specific hemorrhage
Renal hemorrhage
specific hemorrhage
Oral hemorrhage
specific hemorrhage
Epistaxis
specific hemorrhage
specific hemorrhage
optimal factor
dosage(iu/kg)
level (%)
------------------------------------------------------------------Hematoma
20-30
10-15
Hemarthrosis 30-50
15-25
GI tract
40-60
20-30
Retropharyng/tongue 60-100
30-50
Intracranial
80-100
30-50
Retroperitonial
60-100
30-50
Hematuria
30-50
15-25
Minor bleeding
20-30
10-15