Long-term Observation of 87 Cases with Chronic Idiopathic Thrombocytopenic Purpura in Adult / 대한혈액학회지

ABSTRACT

BACKGROUND: Information about the natural history of chronic idiopathic thrombocytopenic purpura (ITP) is still vague and far from complete due to a lack of studies. The purpose of the present study is to define ultimate outcome of adults with chronic ITP by long-term observation of patients. Method : We retrospectively analyzed eighyty-seven ITP patients diagnosed at the Department of Internal Medicine, Kyungpook National University Hospital and Taegu-Hyosung Catholic University Hospital and Taegu-Fatima Hospital between March 1986 and March 1998. RESULTS: Median age was 36 years (range 15 to 74) at the time of diagnosis and median follow-up duration was 46 months (7 to 133). Female to male ratio was 4 : 1. Twenty-four patients who were asymptomatic with stable disease or a platelet count over 50,000/microliter were managed on a wait and see basis. A total of 63 patients received an initial treatment with prednisone (1 mg/kg/day for 1 month). Refractory or relapsed cases underwent splenectomy and/or other therapeutic modalities. An initial complete or partial response to prednisone was observed in 79%. A sustained complete remis- sion (CR) lasting more than 6 months was attained in 11%. Thirty-two refractory or relapsed patients underwent splenectomy. Twenty-two patients (69%) had a CR, with twenty-one (66%) achieving a sustained CR. 5 other cases obtained a partial remission (PR). Long-lasting responses were observed in 3 other case (12 %) following alternative treatment. Five deaths were recorded, but only one was due to thrombocytopenia and the remaining four deaths were unrelated to ITP. Two patients had laboratory features and a clinical history consistent with an autoimmune disease. CONCLUSION: The majority of patients who undergo splenectomy can have a CR for many years. The therapeutic modalities tried as supplements or alternatives to splenectomy all produced some transient remissions but few prolonged recoveries. But the long-term prognosis of chronic ITP is benign even in refractory cases and the development of overt autoimmune diseases is relatively uncommon.