Clinical Symptoms and Response to the Management of Chronic Idiopathic Thrombocytopenic Purpura in Childhood / 대한소아혈액종양학회지

ABSTRACT

BACKGROUND: Idiopathic thromboycytopenic purpura(ITP) is one of the most common acquired bleeding disorders in childhood. The entity of chronic ITP is defined as the persistence of thrombocytopenia for more than 6 months from initial presentation. There are the controversies regarding the diagnosis and management of this disease. The aim of this study is to compare the responses to managements and clinical symptoms of children with chronic ITP. METHODS: We retrospectively analyzed patients with chronic ITP diagnosed and managed between Jan., 1992 and June, 1997. Data from 33 cases were collected. The length of follow-up ranged from 6 months to 5 years. Statistical analysis was done using Jandel Sigmastat software(version 2.0). RESULTS: 1) Mean age is 5.4+/-4.3 years. The ratio of female to male is 0.65. 2) Most common clinical symptoms at presentation were petechiae(81.8%) and mucosal bleeding(42.4%). The patients who presented only petechiae and purpurae were 18 cases(54.5%), both petechiae and mucosal bleeding were 9 cases(27.3%). 3) The platelet count was 43,015+/-39,912/mm3. Bone marrow examinations showed normal or increased megakaryocyte, no abnormal cells and normal cellularity. 4) The prolonged complete response lasting more than 6 months without maintenance therapy was attained in 15.2% of the patients. A continuous complete response was observed in 6.1% to prednisone and IV-gamma-globulin. A prolonged complete response to dexamethasone was in 23.8% and continuous complete response was in 14.3%. The splenectomy was underwent in 3 patients. All of them had a prolonged complete response. One of six patients who were treated with combination chemotherapy had a prolonged complete response. CONCLUSION: This analysis of chronic ITP patients suggests that splenectomy remains the most effective treatment and the next one is high dose dexamethasone therapy. But the number of patients was not enough, more attention should be given to the management of chronic idiopathic thrombocytopenic pupura(ITP).