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ORGAN TRANSPLANTATION: PERSONS WITH CLOTTING DISORDERS Margaret Ragni, MD University of Pittsburgh HEMOPHILIA X-Linked Bleeding Disorder - Antiquity Deficiency of Factor VIII, IX - 1930s Clotting Factor Treatment - 1960s, 1970s Hepatitis B, C - 1970s, 1980s Chronic Liver Disease - 1970s HIV Infection - 1980s Organ Transplant in Hemophilia 1. Bleeding Risk 2. Hepatotoxicity Organ Transplant in Hemophilia 1. Bleeding Risk Poor Fibrin Clot - Lack of Coagulation factor VIII or IX - Decreased synthesis of clotting factors (liver) PoorPlatelet Plug - Thrombocytopenia due to portal hypertension Platelet Defects - Analgesics, antibiotics Organ Transplant in Hemophilia 1. Bleeding Risks Transjugular Percutaneous < 1% (1/178) bleeding complications with biopsy (Ewenstein, 1998) < 1% overall complication rate with transjugular bx (Little, Zajko, 1996) < 1% bleeding complications in hemophilia (Wong et al, 1997) Management of Bleeding Disorder Bleeding History: factor levels Drug History: ASA, NSAIDs Factor levels, PT, APTT, platelet count Treatment: Factor Concentrate for factor deficiency Platelets for portal hypertension DDAVP, Platelets for platelet dysfunction FFP for hepatic synthetic defect Organ Transplant in Hemophilia 2. Hepatotoxicity Liver Function – Site of production of factor VIII or IX Greater liver dysfunction – Multiple hepatitis exposures More frequent drug interactions Organ Transplant in Hemophilia 2. Hepatotoxicity Greater toxicity with antiviral drugs, faster liver dysfunction Potential for antiviral drug intolerance Hepatitis A, B, C, HIV Chronic analgesic, antiviral treatment Liver transplant cures hemophilia SGOT, HCV RNA in HIV(+) Subjects Pittsburgh, Thromb Haemostas 1995;73:1458 Subjects: HIV+ Matched: Age, Date of AIDS Diagnosis At AIDS Diagnosis SGOT (IU/ml) Hemophilic men n = 19 Homosexual men n = 21 HCV Ab(+) HCV RNA (%) (x105 Eq/ml) 152 84.2% 64.02 78 5.9% 3.5 p<.05 p < .001 Hepatotoxocity: Antiviral Therapy Pittsburgh, Blood 1995;85:2337 Subjects: 126 HIV+, CD4 > 200 Rx: AZT + ddI LFT >5XUL Subjects Hemophilic Men Nonhemophilic Men 13/40 (32%) 8/86 (9%) p = .0009 Time to LFT >5XUL 1 year 2 year 25% 34% 11% 11% p = .008 Subjects with Hemophilia 1. Bleeding risk: adequate treatment reduces risk to “usual risk” 2. Hepatotoxicity: monitoring and avoiding potential hepatotoxins reduces risk to “usual risk” 3. Potential Benefit: transplant cures hemophilia Conclusion: No reason to exclude individuals with hemophilia