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In our first experience with TGA we observed its high sensitivity to inhibitor development. Generation of thrombin comes slower in despite of using by-pass agent. Minimum rate of thrombin sufficient for hemostasis is unknown and probably is individual. Duration of the measurement should be prolonged over 90 minutes. The assay has a potential for bypass therapy monitoring.

The finding of 16 bleeding complications in 173 surgical interventions is low (9.2%). Most bleeding complications were seen in patients with vWD type 2B and 3. Patients who underwent major surgery and who received only Minirin had more often bleeding complications. Our evaluation of bleeding incidents in the context of surgery in vWD patients reveals the necessity of special hemostaseologic care for these patients. Substitution therapy should depend on vWD-type and type of surgical intervention.We recommend a therapy with vWF containing FVIII-products for major surgery in vWDpatients. Especially during thoracic surgery, gynecological and otolaryngological surgery a careful peri- and postoperative hemostaseologic management as well as careful coagulation techniques of the surgeon are required.

We successfully eliminated inhibitors in both patients and achieved a complete remission with Rituximab and Prednisone alone (Pat. B) and in combination with Cyclophosphamide (Pat. A). Although Rituximab has been reported to have relatively little toxicity, patient A (70 yrs.) was severely immunocompromized due to an additional dramatic drop of the CD4 cell count, resulting in a life-threatening pulmonary infection and long hospitalization. Our results indicate, that Rituximab therapy has a valuable effect in patients with AH and should be considered in severely affected patients where standard therapy has failed. An optimal initial approach for inhibitor eradication in AH remains to be defined. The additional use of Prednisone and/or Cyclophosphamide might provide a better outcome for a defined patient group. Therefore treatment regimens will have to be patient tailored. The optimum dose, treatment schedule and long-term side effects remain to be elucidated and warrant further investigation.