An Elderly Case of Thrombotic Thrombocytopenic Purpura

A 78-year-old woman was admitted to our hospital because of disorientation and fever on January 21, 1992. Two days before admission she experienced vomiting, anorexia and general malaise. Laboratory examinations on admission disclosed a hemoglobin level of 11.1g/dl and a platelet count of 8, 000/μl. The peripheral blood smear revealed anisocytosis with numerous schistocytes and poikilocytes. Polychromatophilic and nucleated red blood cells were also seen, and the reticulocyte count was 38‰. Her serum lactate dehydrogenase (LDH) value was 2, 977WU and the total serum bilirubin level was 3.5mg/dl with 2.7mg/dl indirect reacting fraction. Serum creatinine was 4.7mg/dl. Her consciousness became semicomatose after a systemic seizure which lasted approximately 15 seconds and her hemoglobin level decreased to 8.5g/dl on hospital day 2. Therefore, we diagnosed her as having thrombotic thrombocytopenic purpura (TTP) because of the presence of all 5 features, that is, thrombocytopenia, microangiopathic hemolytic anemia, fluctuating neurologic abnormalities, renal dysfunction and fever. A plasmapheresis with fresh frozen plasma (FFP) replacement was begun on that day. She was also treated with anti-platelet agents, 80mg/day aspirin, and 300mg/day dipyridamole. Moreover, packed red blood cells (PRC) were infused. While also receiving diphenylhydantoin and phenobarbital to prevent convulsions, status epilepticus developed on day 3. Because of inhibited spontaneous respiration which was an adverse effect derived from diazepam and sodium thiamylal administered intravenously to treat the status epilepticus, an artificial respiration was initiated. Twelve plasmaphereses were performed over 23 days with 25 to 50 units at each procedure and with a total of 375 units of FFP replacement. A total of 9 units of PRC was also transfused. Later, convulsions disappeared on the fifth hospital day and her consciousness gradually became normal, so she was weaned from artificial respiration on day 17. Platelet count rose to over 10×104/μl on day 5, and serum creatinine decreased to the normal range on day 10. Moreover, her indirect bilirubin level also normalized on day 10, but it required about a month for the serum LDH value returned to normal. As sequelae of TTP, mild weakness of the right upper limb and neurogenic bladder were found, and computed tomogram of the brain demonstrated multiple small low density areas. Moreover, thrombophlebitis and edema in the lower limbs due to a bedr