Thrombotic thrombocytopenic purpura.

This case represents a classical presentation of severe TTP. At the time of diagnosis, the patient had a markedly depressed platelet count and evidence of severe hemolytic enemia. Her neurologic symptoms were not ominous at the time of initial presentation but may have worsened rapidly without treatment. She also demonstrates the success of current treatment modalities. A combination of plasma infusion followed by plasmapheresis, anti-platelet agents, corticosteroids, and vincristine was employed. As in most cases of TTP reviewed in the medical literature, it is difficult to sort out which treatment modality was most responsible for the improvement. Since this is such a deadly disease, there is a tendency to treat with a combination of agents in addition to plasmapheresis. Nonetheless, an impression remains that plasmapheresis was most closely associated with her recovery. As an additional comment, the use of plasmapheresis in this patient represented the first use of a pheresis machine that had been obtained in Anchorage, Alaska at the urging of the medical community. Our location, remote from academic centers, has made the use of technology such as plasmapheresis less accessible. The presence of plasmapheresis capabilities in a community setting such as ours raises many questions pertaining to the appropriateness of use of the technology, management of complications, etc.. We plan to carefully monitor these parameters.