2.Hemophilia
description
Transcript of 2.Hemophilia
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