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13 Cards in this Set
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- Back
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hemophilic arthropathy
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from chronic hemoarthrosis
- degenerative cartilage-mediated and inflammatory synovium-mediated components - intra-articular blood = destructive oxygen metabolites (iron-catalysed) 1st affects cartilage 2ndly synovium results in fibrotic and destroyed joint 50% knees, elbows>ankles>shoulders>wrists |
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symptoms of hemarthrosis
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pain
swelling warmth muscle spasm long term effects of recurrent = more serious = damage |
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grading of hemophilia A
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based on FVIII levels
Normal 1 U/mL Severe: <1% normal = spontaneous bleeding symptoms several times per month, require replacements moderate: 1-5%: may bleed spontaneously but less frequent mild >5%-25% - see prolonged post op bleeding |
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chronic arthropathy
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manifested in contraction and severe limitation in motion
characterized by progressive destruction of cartilage and adjacent bone may be prevented by early and prophylactic FVIII replacement therapy; also see improvements with regular exercise (less frequent bleeding, improved joint mobility) |
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hallmark of hemophilia A
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hemoarthrosis
severe: first episode at end of 1st yr of life w/o therapy, returns to normal several days - week after bleeding has stopped each episode followed by acute synovitis, predisposing to further hemorrhages and chronic arthropathy |
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types of bleeding common in hemophilia A
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joint bleeding = hallmark
muscle hematomas - 30% of bleeding episodes intracerebral hemorrhage - common cause mortality; many happen during 1st year of life, before diagnosed hematuria - occurs in up to 2/3s at least once GI (ie ulcers) mucus membranes if severe, if not tests for vWD if get |
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FVIII and vWF
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when FVIII released in plasma, immediately binds to vWF - required for maintaining normal FVIII level in plasma
- prevents premature binding to FIXa before activation - protect FVIII inactivation by protein C, FIXa, FXa ** vWF disease manifests like hemophilia A b/c 2* deficiency in FVIII |
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complication of FVIII replacement therapy
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development of FVIII inhibitory antibodies
- up to 25% pts - become unresponsive to infusions most important risk factor = severity of disease, certain mutations can give high doses to overwhelm Ab can also give procine FVIII activated prothrombin complex concentrate aPTC (Xa*V*PL) - risk DIC or PE rFactor VIIa (longer half life) immunoabsorption prior to FVIII immune tolerance immunoglobins |
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incidence hemophilias
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A: 1 in 5000 male live births
(gen pop 1 in 10,000) B: 1 in 30,000 (gen pop 1 in 60,000) **both x-linked recessive 15-30% spontaneous mutation vWF disease 1 in 1000 |
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intramuscular hemorrhages
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2nd most prevalent bleeding in hemophilias (30%)
- generally resolve w/o complication but large bc bleed along fascial planes - if compartmented, may cause significant compression of vital structures, distal ischemia, gangrene etc - localized tenderness and pain - may be assoc w low grade fevers, ^ LDH, large ecchymoses - bleeding can become life threatening bc large V can be lost in soft tissues of retroperitoneal space -U/S or CT can confirm |
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intracranial hemorrhages in hemophilia
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incidence 2.6-13.8%
kids: almost always preceded by head trauma adults: 50% w/o injury ~30% result in death >50% survivors have long term sequelae symptoms: headache, often w vomiting and seizure, irritability, stiff neck lethargy treat rapidly w admin of XIII or IX concentrates to obtain 100% normal activity |
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hemophilia treatment
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prompt IV replacement therapy with VIII or IX - early = limited bleeding and ensuing tissue damage
std tx 25-40U /kg 3x a week either plasma derived (risk viral infection - non-lipid coated viruses) or recombinant ($$$) also DDVAP, antifibrinolytics, prophylactic therapy |
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DDAVP
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desmopressin acetate
stimulates a 4 fold increase in plasma VIII and vWF by causing release from storage in endothelium and increase production in liver also raises factor XI levels and enhances platelet adhesion and spreading at injury sites mild hemophilia (severe do not have any storage pools of factor VIII) use with antifibrinolytics to reverse the thrombolytic effects generated by the release of t-PA |
