Thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome (TTP/HUS): Clinical presentation in a series of 35 patients

BACKGROUND: Delay in application of massive plasmapheresis is the main unfavorable prognostic factor in TTP/HUS, and it is mainly due to difficulties in diagnosing this rare illness. The aim of our study is to analyze the clinical presentations of TTP/HUS. METHODS: The clinical and laboratory data of 35 patients with TTP/HUS were analyzed, as well as the treatment used and the patients outcome. Time intervals between the beginning of symptoms, the diagnosis and the start of treatment were also recorded. RESULTS: The median age was 37 (range: 11-77) years old and there were 19 (54%) women. The more frequent antecedent was chemotherapy with mitomycin C and 5-fluorouracil (7 cases). The diagnosis of TTP/HUS was reached after a median of 5 (interval 0-293) days, and only 11 (31%) patients were correctly diagnosed in the first medical attendance. Hemolytic anemia and thrombocytopenia was the most frequent clinical presentation (80% of patients). Neurologic signs or symptoms were found initially in 14 patients, and oligoanuria in 4. No patient presented initially with the so called ''clinical pentad'' considered to be typical of TTP/HUS. Plasmapheresis was used in 33 cases. A complete remission was attained in 16, a partial remission in 6, and 13 patients died. CONCLUSION: Due the fickleness of some clinical manifestations considered to be typical of TTP/HUS, these disorders should be considered in patients presenting with acute-onset hemolytic anemia and thrombocytopenia.