HEMOPHILIA A

CATATAN PINGGIR ABS

HEMOSTASIS

PERDARAHAN BERHENTI DAN DARAH TETAP DALAM KEADAAN CAIR

1. KOMPARTEMEN JARINGAN2. KOMPARTEMEN PEMBULUH

DARAH3. KOMPARTEMEN TROMBOSIT4. KOMPARTEMEN PEMBEKUAN5. KOMPARTEMEN FIBRINOLISIS

HEMOSTASIS ABNORMAL

VASCULAR TROMBOSIT PEMBEKUAN

DIATHESIS HEMORRHAGIC

TROMBOSIT

INTEGRITAS VASKULER MENYUMBAT P.DRH CLUMPING

KAPILER VISCOUS METAMORPHOSIS MELEKATKAN JASAD

RENIK

PERDARAHAN

KELAINAN JLH TROMBOSIT BL TIME CL TIME RL

1.Vaskuler N >> N ++

2.Trombosit* @N/<< >> N ++

3. Pembekuan N N >> --

@ N ttp fungsi tdk baik (trombopatia ) * Setiap penyakit yang menyebabkan penurunan tombosit ( ITP,ATP,Leukemia,Aplastik anemia )

INTRINSIC EXTRINSIC

PROTHROMBIN THROMBIN II. PTT

FIBRINOGEN FIBRIN I. TT

III. aPTT

PERDARAHAN• •

• KELAINAN SISTEM PEMBEKUAN

1. THROMBIN TIME FIBRINOGEN?? ( 15-20 DETIK) 2. PROTHROMBIN TIME PROTHROMBIN ?? ( 12-14 DETIK) 3. ACTIVATED PROTHROMBIN TIME VIII a

IX ?? ( 25-40 DETIK)

TT NORMAL PT NORMAL aPTT MEMANJANG

CLOTTING FACTOR ASSAY

F VIII Hemophilia AF IX Hemophilia B F XI Hemophilia CF XII

= Severe hemophilia < 1 IU/dl clotting factor Bleeding spontaneous

= Moderate hemophilia 1 – 5 IU/dl Mild trauma induce bleeding = Mild hemophilia > 5 - 50 IU/dl Significant trauma bleeding

Moderate hemophiliaModerate hemophilia

F VIII

TREATMENT

REPLACEMENT F VIII

ON-DEMAND PROPHYLAXIS

25 IU/Kg BB every other dayDoses of F VIII =% desired(rise in F VIII ) x BW(Kg) x o,5

F VIII

PLASMA RECOMBINANT

HUMAN NON HUMAN @Baxter’Recombinate@Bayer’s Kogenate

Porcine F VIII

INHIBITOR F VIII

Bethesda Inhibitor Assay

LOW ( BIA < 10 )

Increased dose

HIGH ( BIA >10 )

1.Activated Prothrobin Complex Concentrates (APCC)VII,IX,X &Thrombin

2.Porcine F VIII3.Recombinant F VIIIa4.Intravenous Gamma Globulin5.Immune Tolerance6.Plasmapheresis

OTHER TREATMENT MODALITIES

1. DESMOPRESSINEndothelial release of F VIII and vWFMild

2. ANTIFIBRINOLYTIC THERAPYOropharyngeal mucosal bleedingcontraindicated for bleeding in the urinary tract

3. ANALGESICSInterfere with platelet function should be avoided

4. VACCINES HBV

F VIII is synthesized predominantly in the vascular endothelium and is not affected by liver disease

CRYOPRECIPITATE

F VIII 80-100 IUFibrinogen 100-250 mgFibronectin 40-60 mgvWF 40-70 %F XIII 30 %

Original Unit of Plasma

=is no longer used to treat hemophilia A because it is not treated with a virucidal agent=in the treatment of bleeding infantsoften considered because

of its small infusion volume but its not effective in multiple clotting factor deficiency=compatibility test/ Coombs’ test are not necessary for cryoprecipitate

FRESH FROZEN PLASMA

=has been separated with anticoagulant by centrifugation and frozen with 6 hours collection=15 ml/kgBW=efficacious for the treatment of deficiencies F II,V,XI=not recommended for the treatment of deficiencies F VII,VIII,IX

because safer F VII,VIII,IX concentrates available=as replacement therapy along with anticoagulant treatment in patient with thrombosis ( contains several anticoagulant protein : AT III, protein C and S ) =an important ,use of FFP is for rapid reversal of the effects of warfarin in patients who are actively bleeding or who require emergency surgery ( in whom functional deficiencies of FII,VII,IX, and X can not be rapidly reversed by vitamin K=must be ABO compatible with the recipient’red cell;Rh does not be considered