Childhood Acute Immune Thrombocytopenic Purpura in Korea: Multicenter Study of Korean Society of Pediatric Hematology/Oncology / 대한소아혈액종양학회지

PURPOSE: Childhood acute immune thrombocytopenic purpura (ITP) is a benign hematologic disease. Therapy does not affect the natural history of the illness. We evaluated the clinical and laboratory findings, treatment and prognosis of childhood acute ITP in Korea through a retrospective multicenter study. METHODS: We analyzed retrospectively the data of 1, 829 children with acute ITP through survey of 33 hospitals among 43 hospitals in Korea from Sep. 1992 to Aug. 2001. RESULTS: Male to female ratio was 1.3: 1 and the median age at the diagnosis of ITP was 2.9 (0.1 17) years. Median duration of follow up was 6 months. One hundred and forty nine cases of the total 1, 829 patients (8.1%) received no treatment. The initial median platelet count of the non-treated group was 42, 500/mm3. Among the 861 cases who were followed up over 6 months, 315 cases (36.6%) progressed into chronic ITP. Associated with this high rate of chronicity of childhood acute ITP patients in Korea, we must consider the fact that acute ITP patients with fast improvement in the first episode tend not to follow up. Considering that fact, the rate of chronicity becomes 17.2% of the 1, 829 acute ITP patients. The treated group used many kinds of treatment methods. Intravenous immunoglobulins (IVIG) with or without prednisolone (PD) (67.5%) were the most commonly used regimens. In the group treated with IVIG alone, the platelet count began to rise above 50, 000/mm3 at 2.6 days, 100, 000/mm3 at 3.7 days and 150, 000/mm3 at 4.9 days. Four hundred and twenty two cases of the 1, 686 (25.0%) cases followed up after first episode of ITP relapsed. The relapse rate was significantly higher in older patients and in girls than in younger patients and in boys (P< 0.05). The chronicity of ITP statistically increased with age (P< 0.05) and that was the only valuable factor. CONCLUSION: Despite the fact that childhood acute ITP is a pretty common disaese, there is no agreement on the best treatment method for this disease. The establishment of Korean treatment guideline of childhood acute ITP, based on an analysis of multicenters, seems to be needed.

Intravenous Immunoglobulin for Childhood Acute Idiopathic Thrombocytopenic Purura / 대한소아혈액종양학회지

PURPOSE: This study was performed to compare the efficacy and adverse reactions of low dose intravenous immunoglobulin (IVIG) with those of high dose IVIG in childhood acute idiopathic thrombocytopenic purpura (ITP). METHODS: Thirty children who were diagnosed as acute ITP in Seoul National University Children's Hospital from June 2000 to Jan. 2003 were enrolled. IVIG (I.V Globulin S, Green Cross Ltd, Korea) were administered 1 g/kg in low dose group (n=15) and 2 g/kg (400 mg/kg for 5 day or 1 g/kg for 2 days) in high dose group (n=15). RESULTS: 1) The median age of the patients was 4 years (range, 2 months to 13 years) and male to female ratio was 0.5: 1. The median duration of follow-up was 6 months (range, 1 month to 2 year 9 months). 2) The median days to recover the platelet count over 50, 000/muL, 100, 000/muL, 150, 000/muL after IVIG administration were 4, 6, and 7 days in the low dose group and 3, 5, and 5 days in the high dose group. After the initial response, the platelet count decreased below 50, 000/muL in 5 out of 13 patients in the low dose group (the median day, 13) and 6 out of 13 patients in the high dose group (the median day, 21). In 25 patients who were followed over 6 months, 3 out of 13 patients (23.1%) in the low dose group and 4 out of 12 patients (33.3%) in the high dose group were remained as chronic ITP. 3) Adverse reactions related to IVIG administration were observed in 4 out of 15 patients (26.7%) in the low dose group and 5 out of 15 patients (33.3%) in the high dose group. They were fever, headache, nausea and vomiting, and most of them were subsided spontaneously. CONCLUSION: There were no significant difference in the response rate, the relapse rate and the incidence of adverse reactions between the low dose group and the high dose group in childhood acute ITP after IVIG therapy. In respect of the cost and the duration of hospitalization, the low dose IVIG might have some advantages.

Clinical Study of Idiopathic Thrombocytopenic Purpura: Focused on Reticulated Platelet / 대한소아혈액종양학회지

PURPOSE: Immune thrombocytopenic purpura (ITP) is divided into acute and chronic forms. Unfortunately, there have been no known specific laboratory or clinical predictors for the diagnosis of chronic ITP. This study was performed to elucidate the prognostic significance of various clinical and laboratory parameters, including reticulated platelet percentage. METHODS: We retrospectively analyzed 60 patients who were diagnosed as ITP at the Department of Pediatrics, Wonju Christian Hospital from January, 1989 to January, 2001. Various kind of clinical parameters such as age, sex, symptom duration, prior URI history, response to treatment, and laboratory parameters like platelet count at initial presentation, lowest platelet count, duration of thrombocytopenia, initial reticulated platelet percentage, antiplatelet antibody IgG and IgM, antinuclear antibody (ANA), direct and indirect Coombs' test were compared between acute and chronic ITP. RESULTS: Fifteen % of patients (9/60) was chronic ITP. The peak age incidence was from 1 to 3 year of age in both acute (29.4%) and chronic ITP (22.2%). The acute ITP was prevalent in spring season, May and June. There was no difference in the incidence of prior URI history between acute and chronic ITP. Higher proportion of chronic ITP patients (5/9; 55.5%) than acute ITP patients (7/51; 13%) had symptom duration longer than 1 month (P or =1 months duration of presenting symptoms and relapse after 2 months from initial presentation have the propensity of developing chronic ITP. Reticulated platelet percentage could not discriminate acute and chronic ITP. A more accurate detection method should be developed for reticulated platelets.

The Effect of High Dose Dexamethasone in Childhood Acute Idiopathic Immune Thrombocytopenia / 대한소아혈액종양학회지

PURPOSE: For preventing CNS hemorrhage, steroids and high dose immune globulin have been used as the first-line treatments of acute ITP. Most of the patients respond to these treatments with rapid rise of platelet count to safe level compared to patients who received no treatment. Although the exact incidence has not been known, there are patients who are not responding these first-line treatments. Insufficient humoral immune suppression is thought to be the main mechanism of this failure and it has been known that humoral immunity is suppressed only at high dose steroids. Therefore, high dose steroids, especially dexamethasone which has been reported recently as an effective treatment in chronic refractory ITP, can overcome this treatment failure. METHPDS: Among the 62 acute ITP patients who were admitted in pediatric department of Masan Samsung Hospital between March, 1998 and March, 2000, only 8 patients were resistant to high dose immune globulin and conventional dose of prednisone. High dose dexamethasone 25 mg/M(2)/day in three divided doses was given to the 8 treatment failure patients for consecutive 4 days and platelet count was followed at 3, 5 and 7days after beginning of the treatment and then weekly. Only when the initial medication cycle was effective the dexamethasone was given to the patients repeatedly with the same dose and schedule until the resolution of the disease. RESULTS: Platelet counts were increased to safe level (> 50x10(9)/L) within 5 days of the treatment in all the patients with the initial treatment of high dose dexamethasone and this effect was observed in subsequent cycles. The mean days of maintaining platelet count above 20x10(9)/L was 22.0+/-3.1 days. Weight gaining and facial flushing were observed in 57% and 30% of patients but hypertension and glucosuria were not observed. CONCLUSION: These results suggest that high dose dexamethasone may be effective in the treatment of childhood acute ITP who did not respond to conventional dose of prednisone previously.