Twenty years experience with treatment of idiopathic thrombocytopenic purpura in a single department: results in 490 cases.

BACKGROUND

All patients with idiopathic thrombocytopenic purpura (ITP), diagnosed in our Department between June 1970 and December 1989, have been analyzed to evaluate the efficacy of treatment, incidence of spontaneous remission and see whether or not there are some parameters that may be indicative of the clinical outcome of these patients.

PATIENTS AND METHODS

A total of 93 children and 397 adults have been evaluated. Response to treatment was any increase in platelet count to above 30 x 10(9)/1, when the platelet count was < 20 x 10(9)/l, or any doubling of the basal platelet count otherwise. Remission was defined as any platelet count higher than 100 x 10(9)/l lasting for 3 months or longer without treatment. For duration of the response and remission, the last check-up with a favorable platelet count was considered or the midpoint between the last favorable check-up and relapse.

RESULTS AND CONCLUSIONS

In children, short-term responses to prednisone were obtained in 78% of acute patients and 67% of chronic patients, while stable remissions or long-term responses were found in 49% and 17% (p = 0.01). In adults, a short term response to prednisone was obtained in 71% of acute and in 72% of chronic patients, while stable remissions or long-term responses in 30% and 5% (p = 0.000003). In 40/45 children (89%), splenectomy induced a stable remission or a long-term response, while in adults, remissions or a long-term responses were achieved in 100/133 (75%) (p = 0.003). Age below 40 was found to be a significant determinant of a better response (p = 0.0013). Of the 92 evaluable adult patients with borderline thrombocytopenia, 8 developed a severe thrombocytopenia (9%). Spontaneous remissions or spontaneous rises to safe levels of platelet counts were recorded in 9% of adult cases presenting with severe thrombocytopenia: among refractory patients, spontaneous remission occurred significantly more frequently in younger patients (p = 0.003). An overall rate of 19% of adult patients continue to need therapy or have become refractory to any treatment. Among these cases, 7 died from cerebral hemorrhage, 6/7 were older than 50 years. Thus, the overall crude rate of thrombocytopenia-related death for our series of patients was 1.8%.