Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: clinical experience in 108 patients

Article Abstract:

Thrombotic thrombocytopenic purpura is a blood disorder of unknown cause characterized by bleeding into the tissues and anemia. When it occurs in children, it is called hemolytic uremic syndrome. The two diseases are referred to as thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS). Mortality was once virtually universal, but in 1959 exchange transfusions were performed, and mortality declined. Because the disease progresses rapidly, and many different treatments are tried at the same time, the interpretation of effects of the various treatments is very difficult. Plasma infusion therapy was suggested in 1963. From 1979 to 1990, 108 patients were treated for TTP-HUS with prednisone (a corticosteroid), or with plasmapheresis, plasma exchange and prednisone, according to the severity of the illness. Ten patients (9 percent) died, a considerable drop in mortality from earlier years. The results of the study show that the infusion of fresh-frozen plasma alone is insufficient in most patients, but some form of plasma therapy is beneficial, although it is not known how it works. Relapse was frequent with corticosteroids, calling their effectiveness into question, but corticosteroids do seem to be effective in earlier stages of the disease. Splenectomy (removal of the spleen) was performed on six patients, all of whom had adverse reactions, including one death. Splenectomy is no longer performed by the authors to treat TTP-HUS. Antiplatelet therapy was ineffective, and is also no longer administered for this disease. The high rate of relapse raises questions of type and length of therapy. Treatment requires many professional medical personnel, an average of 215 blood donors per patient, and expensive equipment. The large number of donors carries a high risk of disease transmission. (Consumer Summary produced by Reliance Medical Information, Inc.)

Corticosteroids, Adrenocortical hormones, Hemolytic-uremic syndrome, Splenectomy

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Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura

Article Abstract:

Thrombotic thrombocytopenic purpura is a rare disease in which the number of blood-clotting cells (platelets) is reduced, causing bleeding into the tissues. It also causes anemia and neurologic abnormalities. Although the disease is relatively rare, mortality, which used to reach 90 percent, makes it an important disease to treat effectively. In addition, research on the mechanisms of this disease may increase the understanding of other platelet disorders, such as heart attack and stroke. The first real benefit to patients with thrombotic thrombocytopenic purpura occurred when plasma treatments were used, either by exchange or by infusion. If the treatment is effective because it provides a deficient substance, infusion should be effective. If there is not only a deficiency, but also the presence of toxic substances, plasma exchange, which also removes toxic substances, is preferable. A national trial was conducted in which 102 patients were assigned to either infusion or exchange. Side effects, such as hives, nausea, low blood pressure, increased pulse and respiration, dizziness, chills, and edema occurred in many of the patients. Ten patients (nine percent) died; two of these were in the plasma-exchange group. Plasma exchange was found to be more effective for treating thrombotic thrombocytopenic purpura. However, it was still not clear whether the removal of toxic substances or the greater volume of plasma infused during plasma exchange led to the improvement. (Consumer Summary produced by Reliance Medical Information, Inc.)

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Late relapses in patients successfully treated for thrombotic thrombocytopenic purpura

Article Abstract:

Patients who survive a severe episode of thrombotic thrombocytopenic purpura should be followed because they may be at risk of one or more recurrences. Thrombotic thrombocytopenic purpura is a disease of unknown cause marked by abnormally low blood platelet counts accompanied by anemia, fever, neurologic defects and blood vessel obstructions. Researchers followed 63 patients who had been successfully treated for thrombotic thrombocytopenic purpura with either plasma exchange or plasma infusion. There were 30 relapses among 17 of these patients during three to 10 years of follow-up. Nine patients had one recurrence and eight had more than one. The shortest period from initial episode to first relapse was seven months, and the longest was eight years. Data analysis revealed that 36% of patients surviving thrombotic thrombocytopenic purpura will have a relapse within 10 years.

Prognosis, Thrombocytopenic purpura

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