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Stefanus Gunawan
Hemophilia Treatment Center
RD Kandou Hospital
Faculty of Medicine Sam Ratulangi University
CASE 1
Hb 13.1 g/dl
Hct 37.9 %
WBC 8.400/mm3
Plt 589.000/mm
BT 9 (N:3-10 )
CT 6 (N:4-10 )
FURTHER EXAMINATION
No history of bleeding
No family history
PHYSICAL FINDINGS
Compos mentis
7.2 kg; 62 cm
good nutrition
BP 80/50 mmHg
Pulse 100 bpm,
regularly
Respiratory 30 x/m
Temperature
36.6C
Anemic (-)
Upper Lip:
bleeding from
surgical site
Thorax &
Abdomen: no
abnormality
Skin: no bruising
LAB FINDINGS
After consultation:
Hb 11.1 g/dl
Hct 34.9 %
WBC 7900/mm3
Plt 446,000/mm3
PT 12 (10.8-14.4); INR 1.02
aPTT 65 (26.4-37.6)
FVIII 2.3%
Assessment
Mild-bleeding postop labioschizis e.c
Haemophilia A
(moderate)
Treatment
Factor VIII: 20
IU/kgbw/12hr
Tranexamic acid
50mg/kg/dy
Wound care
FOLLOW UP
PASIEN
Target
desired
level
(%)
Weight
(kg)
# Faktor
VIII
needed
(IU)
40
7.2
560
FVIII cost
Balance
Surgery (Minor)
Bleeding
Dy 1-2
2,500,000
10,444,300
#
7,944,300#
LEARNING POINT
Elective surgery
Comprehensive medical history including
family history should be taken
Hemostatic screening test if indicated
CBC,
Blood smear
aPTT, PT
CT not sensitive
Rapaport SI. Blood.1983;61: 229-231
Chee YL et al. Br J Haematol.2008;140:496-504
Laine C et al.Ann Intern Med.2009;151:ITC1-1
10
CASE 2
wound healing
11
PEDIGREE
12
PHYSICAL FINDINGS
Compos mentis
29 kg; 130 cm
good nutrition
BP 100/70 mmHg
Pulse 80 bpm,
regular
Respiratory 24 x/m
Temperature 36C
Anemic (-)
Thorax & Abdomen
no abnormality
detected
Extremity no
hemarthrosis
Genital: phimosis
13
LABORATORY FINDINGS
Hb 13.1 g/dl
Hct 37.9 %
WBC 8400/mm3
Plt 589,000/mm3
PT12.9
(10.8-14.4)
INR 1.02
aPTT 66.7 (26.437.6)
Factor VIII 2%
14
Planning
Advocation to board of director
F VIII replacement pre-post circumcision
15
FOLLOW UP &
FVIII REPLACEMENT SCHEME
16
FOLLOW UP &
FVIII REPLACEMENT SCHEME
Day +1
Bleeding (+)
F VIII 50 IU/kgbw/12 hr (1500IU)
Wound dressing+Tranexamid acid
Day +2
No more bleeding
FVIII 25 IU/kg/12hr, others cont
Day +3
No bleeding discharged
Tranexamic acid p.o only
17
LEARNING POINT
18
CIRCUMCISION IN HEMOPHILIA
WFH 2003
12 hrs prior to
surgery
Oral tranexamic
acid 25-30
mg/kg/day
7
days
2 hrs prior to
surgery
F VIII 20 U/kg
(25 U/kg for
severe cases)*, IV
bolus
Surgery
+ 10 mg/kg
tranexamic acid (or
0.3 mcg/kg DDAVP
infusion for mild
cases)
19
#Hemophilia B, double
doses of factor are used
twice, but DDAVP is not
used
20
Day 11-14:
severe: 10
U/kg/every
other day
moderate: 10
U/kg/every
other day#
21
MILD CASES
Day 4: F VIII
15 U/kg/day
tid
Day 5-7:
10 U/ kg/day
bid + 1 dose
DDAVP
Thereafter,
10 U/kg/every
other day,
once or twice
WFH GUIDELINES
ON MINOR SURGERY (2012)
SUGGESTED PLASMA FACTOR PEAK LEVEL AND DURATION OF ADMINISTRATION
(WHEN THERE IS SIGNIFICANT RESOURCE CONSTRAINT)
Hemophilia A
Desired level
(IU/dL)
Pre-op
40-80
Post-op
20-50
Hemophilia B
Duration (days)
Desired level
(IU/dL)
Duration (days)
40-80
1-5^
20-50
^
1-5^
23
ALTERNATIVE PROTOCOLS
25
26
glue
Gelatin sponge
(Glubran) skin tissue adhesive
Haghpanah S et al. Ann Hematol. 2011;90:463-8.
27
Nama # MR Umur....thn/bln
Kadar factor VIII =.%
Hari
H0
H+1
H+2
H+3
H+4
H+5
H+6
Tanggal
Jam
Tranexamic acid,
iv, bolus
- 30
mnt
Operasi
+ 12
+ 24
+ 36
+ 48
+ 60
+ 72
+ 84
15 mg/kg=...........mg
AHF, iv bolus
25 U/kg=.....U
Dokter yangmerawat
28
Deficit
Circumcision
WFH 2012
(minor surgery)
Sarjito/UGM
Patient
30
120(-290)
17,500,000
225
200
33,600,000
28,800,000
19,859,600
-8,940,400
29
WFH RECOMMENDATIONS:
CIRCUMCISION OF PWH
Informed consent
Blood products (FC, PRC, FFP, etc)
Other medications (tranexamic acid, DDAVP, analgesics,
antibiotics, etc)
Generally risk of postoperative bleeding in PWH 15-20%
30
CASE 3
31
HISTORY
Head trauma
Hit bedhandle
Dy
Vomit
Weakness
-7
right limb
Hospitalised
Ampana
Dy
Less conscious
Seizure
-6
R/FVIII 750IU
once
Arrived
RDK
Dy 0Unconsciou
s
No seizure
32
PEDIGREE
33
PHYSICAL FINDINGS
34
PHYSICAL FINDINGS
Physiologic reflexes /N
Pathologic reflex: +/+
Spastic (-), clonus (-)
35
LABORATORY FINDINGS
Hb 13.1 g/dl
Hct 38.2 %
WBC 12,900/mm3
Plt 560,000/mm3
PT 12.3 (10.8-14.4); INR 0.97
aPTT 31.0 (26.4-37.6)
Factor VIII <1%
36
Intracerebral haemorrhage
frontotemporoparietalis sinistra, subdural
haemorrhage, subarachnoid haemorrhage
37
1L/m
IV NaCl 0.45% in D5% 50ml/m
F VIII 2 x 1500IU (50 IU/kg/12hr)
Tranexamid acid 3 x 750 mg, IV
Furosemid
1 x 20 mg, IV
Mannitol 75 cc in 30 3x/dy
Cefotaxime
3 x 1 gr, IV
Paracetamol 3 x500 mg prn, IV
38
Dy+1-+3
GCS 10
R/ FVIII 50
IU/kg/12hr
others cont
Dy +4-+7
GCS 11
R/ FVIII 25
IU/kg/12hr
Mannitol &
Frusemide stop
Rehabilitation
DY +8-+9
GCS 12 no fever
R/ FVIII 20
IU/kg/12hr
Cefotaxime
stop
39
Dy +10-+13
GCS 15
Motoric Right
R/ FVIII 10
IU/kg/12hr
Dy +14
Status quo
discharged by
parents own
40
PASIEN
Target
desired
level
(%)
Weight
(kg)
50-80
30-50
20-40
30
Deficit
CNS Bleeding
Dy 1-3
Dy 4-7
Dy 8-14
19,800
95.040.000
19.859.600
41
-75.180.400
LEARNING POINTS
42
43
WFH GUIDELINES
ON CNS BLEEEDING (2012)
SUGGESTED PLASMA FACTOR PEAK LEVEL AND DURATION OF ADMINISTRATION
(WHEN THERE IS NO SIGNIFICANT RESOURCE CONSTRAINT)
Hemophilia A
Hemophilia B
Desired level
(IU/dL)
Duration (days)
Desired level
(IU/dL)
Duration (days)
Initial
80-100
1-7
60-80
1-7
Maintenance
50
8-21
30
8-21
44
WFH GUIDELINES
ON CNS BLEEDING (2012)
SUGGESTED PLASMA FACTOR PEAK LEVEL AND DURATION OF ADMINISTRATION
(WHEN THERE IS SIGNIFICANT RESOURCE CONSTRAINT)
Hemophilia A
Hemophilia B
Desired level
(IU/dL)
Duration (days)
Desired level
(IU/dL)
Duration (days)
Initial
50-80
1-3
50-80
1-3
Maintenance
30-50
4-7
30-50
4-7
20-40
8-14
20-40
8-14
45
Neurosurgery:
Failure
of conservative-replacement
therapy
Severe-life threatening ICH
Cermelj M et al.Haemophilia. 2004;10:405-7
Wu XJ et al. Br J Neurosurg. 2009;23:455-7
46
Klinge et al:
Haemophilia 2008;14:952-5
1960-1991: 29.2%
2001: 18.2%
2003: 8.6%
De Tezanos-Pinto. Haemostasis.1992;22:259-67
Nuss. Am J Hematol.2001;68:37-42
Antunes. Haemophilia.2003;9:573-7
47
WFH RECOMMENDATIONS:
ICH IN PWH
A medical emergency. Treat first
before evaluating
All post-traumatic head injuries,
confirmed or suspected, &
significant headaches must be
treated as intracranial bleeds
Do not wait for further symptoms
to develop or for laboratory or
radiologic evaluation
48
THANK YOU
51