Abstract
A 62-year-old man with no evidence of a bleeding tendency or a family history of hereditary bleeding disease began receiving dialysis therapy for the treatment of end-stage renal disease arising from benign nephrosclerosis in July 2009 at another hospital; he was subsequently referred to our hospital for regular dialysis treatment. For several years, no difficulties in treating this patient were encountered. In July 2012, however, we began to note that a longer period of time seemed to be required for the patient to stop bleeding from the vascular access puncture sites after the completion of his dialysis treatments. On one occasion, the application of pressure hemostasis for more than one hour was required before the bleeding could be stopped and the patient was able to go home. However, the patient subsequently returned to the hospital and was admitted because of a massive subcutaneous hemorrhage extending from his left upper arm to the chest due to re-bleeding from the puncture sites. During the period of hospitalization, since intractable subcutaneous upper arm bleeding occurred on repeated occasions, we examined his bleeding and coagulate functions. His activated partial thromboplastin time was markedly extended and his factor VIII activity level was significantly decreased, with the presence of 10 Bethesda units/mL of factor VIII inhibitory activity. Accordingly, we diagnosed the patient as having acquired hemophilia A. Considering his old age and his uremic immune-compromised state, the early cessation of steroid treatment was desired. Consequently, we treated him using bypass therapy and the removal of inhibitory antibodies in combination with a low-dose, short-term steroid administration period. Double filtration plasmapheresis (DFPP) was used to remove the antibodies effectively. After 6 sessions of DFPP, the inhibitors disappeared and his hemostasis eventually recovered to normal. We concluded that DFPP is an effective modality for treating patients with acquired hemophilia A, especially those with an immune-compromised state such as uremia, when used in combination with low-dose steroid administration.
