HEMOFILIA

SUB BAG. HEMATO – ONKOLOGI

ANAK FK USU
• HEREDITER – X LINKED RECESSIVE
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• JENISNYA:
1. HEMOFILIA A ( HEM. KLASIK )
2. HEMOFILIA B (CHRISTMAS DISEASE )
3. HEMOFILIA C ( ROSENTHAL SYND.)
• HEM. KLASIK:
- BERAT : AKTIFITAS FAKTOR
BEKUAN 1%
- SEDANG : AKTIFITAS FAKTOR
BEKUAN 1 – 5 %
- RINGAN : AKTIFITAS FAKTOR
BEKUAN 5 – 20 %
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• SKRINING PERDARAHAN:
- MASA PERDARAHAN
- MASA PEMBEKUAN
- JUMLAH TROMBOSIT
- RUMPLE LEEDE
• PEMERIKSAAN LANJUTAN:
- a P.T.T
- P.T
- T.T
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- PENGUKURAN KADAR FAKTOR
PEMBEKUAN TERTENTU
• PENATALAKSANAAN:
1. BILA ADA PERDARAHAN ATAU
AKAN DILAKUKAN TINDAKAN YG
DPT MENIMBULKAN PERDARAHAN
A. KRIOPRESIPITAT
- UTK HEMOFILIA A
- 1 BAG = 100 U FAKTOR VIII
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B. FRESH FROZEN PLASMA
- PD HEMOFILIA B
- 1 U AHG = 1 ML PLASMA
= 2,5 ML DARAH
C. PLASMA SEGAR
D. DARAH SEGAR
2. TINDAKAN PROFILAKSIS
- F. VIII 3 X SEMINGGU  ADA YG
MEMBERI 1 X SEMINGGU
- PEMBERIAN EACA  ?
- KORTIKOSTEROID
 3. PENGOBATAN PSIKIATRI 5
4. HOME THERAPY
• GUIDELINES FOR REPLACEMENT
THERAPY BEFORE AND AFTER
ELECTIVE SURGERY:
A. BEFORE PROCEDURE:
1. COMPLETE COAGULATION WORKUP
2. INCUBATED TEST FOR
INHIBITORS
3. CALCULATE NEEDS & STOCKPILE
THERAPEUTIC MATERIAL
 4. DETERMINE HALF-LIFE OF 6
THERAPEUTIC MATERIAL
5. COMPLETE BLOOD, PLATELET,
AND RETICULOCYTE COUNTS
6. DETERMINE RED CELL TYPE
B. GENERAL SURGICAL PROCEDURE:
1. MINOR
A. GIVE DOSE CALCULATED TO
BRING PATIENT’S PLASMA
LEVEL TO 100 % 1 HOUR
BEFORE PROCEDURE (50
UNITS/KG)
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B. MAINTAIN PLASMA LEVEL
ABOVE 60 % FOR 4 DAYS
C. MAINTAIN PLASMA LEVEL
ABOVE 20 % FOR SUBSEQUENT
4 DAYS
D. ASSAY DAILY BEFORE
ADMINISTRATION
2. MAJOR
A. GIVE DOSE CALCULATED TO
BRING PATIENT’S PLASMA
LEVEL TO 100 % 1 HOUR BEFORE
PROCEDURE ( 50 UNITS/KG )
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B. MAINTAIN PLASMA LEVEL ABOVE 60
% FOR 4 DAYS
C. MAINTAIN PLASMA LEVEL ABOVE 40
% FOR NEXT 4 DAYS OR UNTIL ALL
DRAINS & SUTURES REMOVED
D. ASSAY DAILY BEFORE
ADMINISTRATION
C. ORTHOPEDIC PROCEDURE:
1. GIVE DOSE CALCULATED TO
BRING PATIENT’S PLASMA LEVEL
TO 100 % 1 HOUR BEFORE
PROCEDURE ( 50 UNITS/KG )
 2. MAINTAIN PLASMA LEVEL 9
ABOVE 80 % FOR 4 DAYS ( 40
UNITS/KG 3 TIMES DAILY )
3. MAINTAIN PLASMA LEVEL
ABOVE 40 % FOR SUBSEQUENT 4
DAYS (40 UNITS/KG 2 TIMES DAILY )
4. ASSAY DAILY BEFORE
ADMINISTRATION 5.
IF PATIENT IS IN CAST,
DISCONTINUE REPLACEMENT
UNTIL REHABILITATION PROGRAM
 6. IF PATIENT IS NOT IN CAST,10
MAINTAIN PLASMA LEVEL ABOVE
20 % FOR AMBULATION
7. FOR REHABILITATION PROGRAM
MAINTAIN PLASMA LEVEL ABOVE
10 % FOR 3 WEEKS
D. DENTAL PROCEDURE:
1. GIVE EACA 100 MG/KG 4 HOURS
BEFORE SURGERY
2. GIVE CLOTTING FACTOR DOSE
CALCULATED TO BRING PLASMA
LEVEL TO 100 % 1 HOUR BEFORE
PROCEDURE
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3. CONTINUE EACA 100 MG/KG
EVERY 6 HOURS FOR 10 TO 14 DAYS
4. REPEAT REPLACEMENT THERAPY
IN 3 DAYS IF PROCEDURE IS
EXTENSIVE