[Thrombotic thrombocytopenic purpura--still a therapeutic problem. Case report of a patient on long-term therapy]

Thrombotic thromboctopenic purpura is a rare multisystemic life threatening disease the treatment of which is still a serious problem. The most successful therapy is plasmapheresis where the whole plasma volume of the patient is replaced by fresh frozen plasma or cryosupernatant. The authors describe a 34-year-old patient with the chronic relapsing form of the disease and repeated cerebrovascular attacks with an ischaemic genesis with developed organic psychosyndrome. The only effective treatment of the patient are exchange plasmaphereses replacing plasma by cryosupernatant after the patient became refractory to fresh frozen plasma. The authors describe the clinical development of the disease, its treatment by plasmaphereses and mention various ways of a venous approach and associated problems. Ensuring a venous approach in patients with this disease is of vital importance and in the terminal stage of the disease it is extremely difficult. A solution, though temporary, is implantation of a vascular prosthesis, Diastat. The functioning of the implant in this disease is however greatly threatened by the development of thrombotic occlusions.