Summary
At present, the future of patients with hemophilia A may be regarded with more optimism due to advances in diagnosis and management which have occurred in recent years. However, a number of problems are still unresolved and others are now emerging against this optimistic background. The development of an inhibitor specifically destroying factor VIII coagulant activity renders the management at best difficult. It has been recognized recently that multitransfused hemophiliacs have a high incidence of abnormal liver function tests, and there is preliminary evidence that these are the expression of chronic active liver disease. This review is focused on these pitfalls of hemophilia care and emphasizes the recent developments in these fields and their impact on the pattern of life of hemophiliacs.
References
Abildgaard C. F., Penner J. A., Watson-Williams E. J.: Anti-inhibitor coagulant complex (Autoplex) for treatment of factor VIII inhibitors in hemophilia — Blood56, 978, 1980.
Aledort L. M.: Personal communication, 1981.
Allain J. P., Krieger G. R.: Prothrombin complex concentrate in treatment of classical hemophilia with factor VIII antibody. (Letter) — Lancetii, 1203, 1975.
Anderle K.: The clinical application of FEIBA Immuno in factor VIII inhibitor patients — Thrombos. Haemost.38, 347, 1977. (Abstract).
Berk P. D., Jones E. A., Plotz P. H.: Corticosteroid therapy for chronic active hepatitis. (Editorial) — Ann. intern. Med.85, 523, 1976.
Bloom A. L.: Clotting factor concentrates for resistant haemophilia — Brit. J. Haemat.40, 21, 1978.
Brackmann H. H.: Personal communication, 1981.
Brackmann H. H., Gormsen J.: Massive factor-VIII infusion in hemophiliac with factor-VIII inhibitor, high responder. (Letter) — Lancetii, 933, 1979.
Count R. B.: Serum transaminase and HBAb in hemophiliacs treated exclusively with cryoprecipitate. In:Fratantoni J. C., Aronson D. L. (Eds): Unsolved therapeutic problems in hemophilia. DHEW Publ. no. (NIH)-77-1089, Washington DC, Government Printing Office, 1976; p. 77.
Craske J., Dilling N., Stern D.: An outbreak of hepatitis associated with intravenous injection of factor-VIII concentrate — Lancetii, 221, 1975.
Edson J. R., McArthur J. R., Branda R. F., McCullough J. J., Chou S. N.: Successful managements of a subdural hematoma in a hemophiliac with an anti-factor VIII antibody — Blood41, 113, 1973.
Enck R. E., Betts R. F., Brown M. R., Miller G.: Viral serology (hepatitis B virus, cytomegalovirus, Epstein-Barr virus) and abnormal liver function tests in transfused patients with hereditary hemorrhagic diseases — Transfusion19, 32, 1979.
Gerety R. J., Schweitzer I. L.: Viral hepatitis type B during pregnancy, the neonatal period and infancy — J. Pediat.90, 368, 1977.
Hasiba V. W., Spero J. A., Lewis J. H.: Chronic liver dysfunction in multitransfused hemophiliacs — Transfusion17, 490, 1977.
Hilgartner M. W., Giardina P.: Liver dysfunction in patients with hemophilia — Scand. J. Haemat.30 (Suppl.), 6, 1977.
Hruby M. A., Schauf V.: Transfusion related short-incubation hepatitis in hemophilic patients — J. Amer. med. Ass.240, 1355, 1978.
Hultin M.: Activated clotting factors in factor IX concentrates — Blood54, 1028, 1979.
Kasper C. H., Feinstein D. I.: Rising factor VIII inhibitor titers after Konyne factor IX complex. (Letter) — New Engl. J. Med.295, 505, 1976.
Kasper C. H., Kipnis S. A.: Hepatitis and clotting factor concentrates. (Letter) — J. Amer. med. Ass.221, 510, 1972.
Kelly P., Penner J. A.: Antihemophilic factor inhibitors. Management with prothrombin complex concentrates — J. Amer. med. Ass.236, 2061, 1976.
Lechner K., Nowotny C. H., Krinninger B., Zegner M., Deutsch E.: Effect of treatment with activated prothrombin complex concentrate (FEIBA) on factor VIII-antibody level — Thrombos. Haemost.40, 478, 1978.
Lesesne H. R., Morgan J. E., Blatt P. M., Webster W. P., Roberts H. R.: Liver biopsy in hemophilia A — Ann. intern. Med.86, 703, 1977.
Levine P. H., McVerry B. A., Attock B., Dormandy K. M.: Health of the intensively treated hemophiliac, with special reference to abnormal liver chemistries and splenomegaly — Blood50, 1, 1977.
Lusher J. M., Shapiro S. S., Palascak J. E., Rao A. V., Levine P. H., Blatt P. M. and theHemophilia Study Group: Efficacy of prothrombin-complex concentrates in hemophiliacs with antibodies to factor VIII. A multicenter therapeutic trial — New Engl. J. Med.303, 421, 1980.
MacGrath K. M., Lilleyman J. S., Triger D. R., Underwood J. C. E.: Liver disease complicating severe hemophilia in childhood — Arch. Dis. Childh.55, 537, 1980.
Mannucci P. M., Bader R., Ruggeri Z. M.: Concentrates of clotting factor IX. (Letter) — Lanceti, 41, 1976.
Mannucci P. M., Capitanio A., Del Ninno E., Colombo M., Pareti F. I., Ruggeri Z. M.: Asymptomatic liver disease in hemophiliacs — J. clin. Path.28, 620, 1975.
Mannucci P. M., Colombo M., Rizzetto M.: Non-progressive course of non-A, non-B chronic hepatitis in hemophiliacs — To be published, 1982.
Mannucci P. M., Federici A., Viganò S., Cattaneo M.: Multiple dental extractions with a new prothrombin complex concentrate in two patients with factor VIII inhibitors — Thrombos. Res.15, 359, 1979.
Mannucci P. M., Ronchi G., Rota L., Colombo M.: A clinicopathological study of liver disease in hemophiliacs — J. clin. Path.31, 779, 1978.
Mannucci P. M., Ruggeri Z. M., Pareti F. I., Capitanio A.: 1-Deamino-8-D-arginine vasopressin: a new pharmacological approach to the management of hemophilia and von Willebrand’s disease — Lanceti, 869, 1977.
Mariani G., Romoli D., Salvitti C., Avvisati G., Hassan H. J., Mazzucconi M. G.:In vivo activation of the extrinsic pathway by FEIBA — Thrombos. Haemost.42, 365, 1979. (Abstract).
Onder O., Hoyer L.: Factor VIII coagulant antigen in factor IX complex concentrates — Thrombos. Res.15, 569, 1979.
Penner J., Kelly P.: Factor VIII inhibitors: therapeutic response to prothrombin complex concentrates — Thrombos. Haemost.38, 339, 1977.
Plotz P. H.: Asymptomatic chronic hepatitis. (Editorial) — Gastroenterology68, 1629, 1975.
Preston F. E., Dinsdale R. C. W., Sutcliffe D. S., Bardhan G., Wyld P. H., Hamlyn J. F.: Factor VIII inhibitor by-passing activity (FEIBA) in the management of patients with factor VIII inhibitor — Thrombos. Res.11, 643, 1977.
Preston F. E., Triger D. R., Underwood J. C. E., Bardhan G., Mitchell V. E., Stewart R. M., Blackburn E. K.: Percutaneous liver biopsy and chronic liver disease in hemophiliacs — Lancetii, 592, 1978.
Schimpf K. L., Zimmermann K.: Hepatitishäufigkeit, serologische Befunde und Leberhistologie nach Therapie schwerer hämorrhagischer Diathesen mit Gerinnungsfaktoren-Konzentraten. In:Schimpf K. L. (Ed.): Fibrinogen, fibrin and fibrin glue. Side effects of therapy with clotting factor concentrates. F. K. Schattauer, Stuttgart and New York, 1980; p. 229.
Schimpf K. L., Zimmermann K., Rudel J., Thanner G., Zeltsch P.: Result of liver biopsies, rate of icteric hepatitis and frequency of anti-HBs and HBs-antigen in patients of the Heidelberg hemophilia centre — Thrombos. Haemost.38, 340, 1977. (Abstract).
Seef L. B., Hoofnagle J.: Acute and chronic liver disease in hemophilia. In:Fratantoni J. C., Aronson D. L. (Eds): Unsolved therapeutic problems in hemophilia. DHEW Publ. no. (NIH)-77-1089, Washington DC, US Government Printing Office, 1976; p. 61.
Sjamsoedin-Visser L. J. M., Heisnen L., Mauser-Bunschoten E. P., Van Geijlswijk J. L., Houwelingen H., Asten P., Sixma J. J.: Clinical trial of activated prothrombin-complex in hemophilia A with inhibitor — To be published, 1981.
Spero J. A., Lewis J. H., Fisher S. E., Hasiba V., Van Thiel D. H.: The high risk of chronic liver disease in multitransfused juvenile hemophilic patients — J. Pediat.94, 875, 1979.
Spero J. A., Lewis J. H., Van Thiel D. H., Hasiba V., Rabin B.: Asymptomatic structural liver disease in hemophilia — New Engl. J. Med.298, 1373, 1978.
Stenjberg S., Jorgensen J.: Activated FIX concentrate (FEIBA) used in the treatment of hemophilic patients with antibody to FVIII — Acta med. scand.203, 471, 1978.
Sultan Y., Brovet J. C., Debre C.: Treatment of inhibitors to factor VIII with activated prothrombin concentrate — New Engl. J. Med.291, 1974, 1974.
Yolken R. H., Hilgartner M. W.: Prothrombin complex concentrates in hemophiliacs with factor VIII inhibitors — Amer. J. Dis. Child.132, 291, 1978.
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Mannucci, P.M. Problems in hemophilia therapy. La Ricerca Clin. Lab. 11, 301–311 (1981). https://doi.org/10.1007/BF02909029
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DOI: https://doi.org/10.1007/BF02909029