Abstract
Clinical cure of hemophilia A by orthotopic liver transplantation has been reported in 11 cases. We describe the first successful Italian case. A 27-year-old man had cirrhosis caused by previous infections with the hepatitis B, C and D viruses following life-long treatment with factor VIII concentrates made from large plasma pools. He was, however, seronegative for the human immunodeficiency virus. In the year before transplantation, life-threatening gastrointestinal bleeding due to severe esophageal varices required a large transfusion regimen (on average, 13 bags of red cell concentrates and 35,000 U of factor VIII/week). To perform orthotopic liver transplantation 8,000 U of factor VIII were given during surgery together with 10 bags of red cells and 11 of fresh-frozen plasma. Intraoperative bleeding was not different from that of non-hemophilic patients undergoing orthotopic liver transplantation. No additional factor VIII was used after transplantation and factor VIII levels in plasma were always above 50 U/dl, reaching the highest value of 184 U/dl on day 4 post transplantation. He was discharged from hospital 10 weeks after trans-plantation with factor VIII levels of 68 U/dl. All virological markers are currently negative, except anti-hepatitis C virus antibodies. In this patient orthotopic liver trans-plantation was a life-saving treatment for end-stage cirrhosis and a cure for hemophilia A.
| Original language | English |
|---|---|
| Pages (from-to) | 44-46 |
| Number of pages | 3 |
| Journal | International Journal of Clinical & Laboratory Research |
| Volume | 25 |
| Issue number | 1 |
| DOIs | |
| Publication status | Published - Mar 1995 |
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Keywords
- Coagulation factors
- Factor VIII/von Willebrand factor
- Hemophilia
- Liver transplantation
ASJC Scopus subject areas
- Clinical Biochemistry
Cite this
Orthotopic liver transplantation in a patient with severe hemophilia A : a life-saving treatment for the first Italian case. / Federici, A. B.; Mannucci, P. M.; Stabile, F.; Rossi, G.; Piseddu, G.
In: International Journal of Clinical & Laboratory Research, Vol. 25, No. 1, 03.1995, p. 44-46.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Orthotopic liver transplantation in a patient with severe hemophilia A
T2 - a life-saving treatment for the first Italian case
AU - Federici, A. B.
AU - Mannucci, P. M.
AU - Stabile, F.
AU - Rossi, G.
AU - Piseddu, G.
PY - 1995/3
Y1 - 1995/3
N2 - Clinical cure of hemophilia A by orthotopic liver transplantation has been reported in 11 cases. We describe the first successful Italian case. A 27-year-old man had cirrhosis caused by previous infections with the hepatitis B, C and D viruses following life-long treatment with factor VIII concentrates made from large plasma pools. He was, however, seronegative for the human immunodeficiency virus. In the year before transplantation, life-threatening gastrointestinal bleeding due to severe esophageal varices required a large transfusion regimen (on average, 13 bags of red cell concentrates and 35,000 U of factor VIII/week). To perform orthotopic liver transplantation 8,000 U of factor VIII were given during surgery together with 10 bags of red cells and 11 of fresh-frozen plasma. Intraoperative bleeding was not different from that of non-hemophilic patients undergoing orthotopic liver transplantation. No additional factor VIII was used after transplantation and factor VIII levels in plasma were always above 50 U/dl, reaching the highest value of 184 U/dl on day 4 post transplantation. He was discharged from hospital 10 weeks after trans-plantation with factor VIII levels of 68 U/dl. All virological markers are currently negative, except anti-hepatitis C virus antibodies. In this patient orthotopic liver trans-plantation was a life-saving treatment for end-stage cirrhosis and a cure for hemophilia A.
AB - Clinical cure of hemophilia A by orthotopic liver transplantation has been reported in 11 cases. We describe the first successful Italian case. A 27-year-old man had cirrhosis caused by previous infections with the hepatitis B, C and D viruses following life-long treatment with factor VIII concentrates made from large plasma pools. He was, however, seronegative for the human immunodeficiency virus. In the year before transplantation, life-threatening gastrointestinal bleeding due to severe esophageal varices required a large transfusion regimen (on average, 13 bags of red cell concentrates and 35,000 U of factor VIII/week). To perform orthotopic liver transplantation 8,000 U of factor VIII were given during surgery together with 10 bags of red cells and 11 of fresh-frozen plasma. Intraoperative bleeding was not different from that of non-hemophilic patients undergoing orthotopic liver transplantation. No additional factor VIII was used after transplantation and factor VIII levels in plasma were always above 50 U/dl, reaching the highest value of 184 U/dl on day 4 post transplantation. He was discharged from hospital 10 weeks after trans-plantation with factor VIII levels of 68 U/dl. All virological markers are currently negative, except anti-hepatitis C virus antibodies. In this patient orthotopic liver trans-plantation was a life-saving treatment for end-stage cirrhosis and a cure for hemophilia A.
KW - Coagulation factors
KW - Factor VIII/von Willebrand factor
KW - Hemophilia
KW - Liver transplantation
UR - http://www.scopus.com/inward/record.url?scp=0029196566&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0029196566&partnerID=8YFLogxK
U2 - 10.1007/BF02592576
DO - 10.1007/BF02592576
M3 - Article
VL - 25
SP - 44
EP - 46
JO - Ricerca in Clinica e in Laboratorio
JF - Ricerca in Clinica e in Laboratorio
SN - 0940-5437
IS - 1
ER -