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.

A Case Of Common Variable Immunodeficiency with Fatal Measles

Common variable immunodeficiency(CVID) is a heterogeneous immunodeficiency syndrome characterized by hypogammaglobulinemia, recurrent bacterial infections, and a variety of immunological abnormalities. We report a 10-year-old boy diagnosed with CVID and a fatal case of measles. He was admitted for fever, cough, conjunctival injection with purulent discharge and maculopapular rash with some scattered pinpoint sized petechial lesions on the whole body for a duration of 5 days. Laboratory findings showed hypogammaglobulinemia [IgG 617 mg/dL(639-1,349 mg/dL), IgA 8.5 mg/dL(70-312 mg/dL), IgM 34.1 mg/dL(56-352 mg/dL)] and anti-measles antibody and anti HIV antibody were negative. He was treated with intravenous immunoglobulin 700 mg/kg on admission. Radiologic findings progressed from hyperaeration to fluffy margined interstitial and alveolar infiltrations on both lung fields. In spite of supportive and ventilator therapy, his pneumonia progressed to acute respiratory distress syndrome and he expired.

Outcome after Treatment of Retinoblastoma

PURPOSE: Current treatment in retinoblastoma aims to save life and preserve useful vision by using combined modality therapy. This study was performed to determine the outcome after treatment of retinoblastoma. METHODS: Medical records of all cases of retinoblastoma seen at Seoul National University Children's Hospital from September 1986 to April 1995 were reviewed. During this period, the treatment method was consistent for the patients with retinoblastoma as follows; for intraocular disease with visual potential, nonsurgical treatment consisted of radiation therapy and/or light coagulation, cryotherapy was considered, followed by enucleation if there was no response, and for intraocular disease without visual potential and extraocular disease, enucleation of those eyes that had severe involvement followed by radiation therapy and/or chemotherapy (systemic or intrathecal) was done according to the clinical pathologic staging. RESULTS: In 58 patients, 6 patients died and the 3-year survival rate was 83%. There was no difference in survival rate between 47 unilateral and 11 bilateral cases, which were 80% and 91%, respectively (P=0.790). The survival rate of 74% for 16 extraocular disease was lower than that of 87% for 42 intraocular disease and the difference of the survival rate between these two groups has shown borderline statistical significance (P=0.056). The 3-year event free survival rate was 69% in overall patients. Complications following therapy included infection due to bone marrow suppression, cardiomyopathy, and orbital contracture. One case of osteosarcoma has developed as a second malignant neoplasm. CONCLUSIONS: To improve the survival of retinoblastoma, effective therapy for extraocular disease and prevention of extraocular extension are important. Supportive care to minimize the complications of therapy and long-term follow-up for second malignant neoplasm are also needed.