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Angeborene Koagulopathien am Beispiel der Hamophilie A und B, Hemmkorperhamophilie Oldenburg J; Barthels MHamostaseologie 2008[Dec]; 28 (5): 335-47An overview is given on the clinical picture and therapy of the haemophilias A and B as an example for congenital coagulopathies. The survey deals with the special problems of today's "treated haemophilia" and its recommendations on diagnosis and treatment regimens. A special focus is put on the pathogenesis of the haemophilic joint arthropathy, that is affecting most haemophiliacs earlier or later during life. Safe plasma derived and recombinant clotting factor concentrates allow an efficient on-demand and also a prophylactic treatment for the prevention of bleeding sequelae. As a consequence life quality and life expectancy of today born haemophiliacs have nearly normalised. The problems of haemophilia with inhibitors and future therapeutic options are discussed. The development of inhibitors at start of therapy with factor VIII concentrates represent the nowadays most serious complication of haemophilia treatment. The only way to a long lasting eradication of the inhibitor ist the induction of immune tolerance that can be achieved by various protocols. A successful immune tolerance therapy (ITT) is the precondition for a regular prophylactic treatment regime. The Guidelines of the Federal Chamber of Physicians, the German recommendations for ITT and the consensus recommendations of an international working group provide an important lead for the conduction of immune tolerance therapy.|Blood Coagulation Disorders, Inherited/blood/*therapy[MESH]|Blood Coagulation Disorders/blood/*therapy[MESH]|Blood Platelets/physiology[MESH]|Female[MESH]|Hemophilia A/blood/complications/diagnostic imaging/*therapy[MESH]|Hemophilia B/blood/*therapy[MESH]|Humans[MESH]|Infant, Newborn[MESH]|International Normalized Ratio[MESH]|Joint Diseases/etiology[MESH]|Partial Thromboplastin Time[MESH]|Pregnancy[MESH]|Prenatal Diagnosis[MESH]|Radiography[MESH] |
