Hepatopathy-thrombocytopenia vs infection-induced hemophagocytic syndrome in Wilms' tumor: a case report.

A 3-year-old boy with Wilms' tumor, post operative left nephrectomy stage, had HTS on day 99 of the combined chemotherapy which lasted for more than 20 days. He had severe respiratory distress due to a moderate amount of ascites and marked pleural effusion. Because of high fever, thrombocytopenia and marked hemphagocytosis in the bone marrow, he received IVIG for 2 days. Normal platelet count and markedly decreased pleural fluid were attained within 3 days. He subsequently tolerated full doses of combined chemotherapeutic agents with an additional one (doxorubicin). In cases of HTS, IAHS should be suspected. The bone marrow should be done and treatment accordingly so that there is no need to decrease, the dosage of chemotherapeutic agents afterwards.

Results of the splenectomy in children with thalassemia.

Sixty-nine children with beta-thalassemia/E disease, hemoglobin H disease and beta-thalassemia major who were followed for at least 2 years before and after splenectomy were studied retrospectively for the results of the splenectomy. It was found that. 1. The hematocrit increased significantly in beta-thalassemia/E from 18 to 22 per cent, in hemoglobin H disease from 21 to 34 per cent and in beta-thalassemia major from 14-15 to 18-19 per cent. 2. The requirement of blood transfusions per year decreased significantly from 6 to 2 times in beta-thalassemia/E, 10 to 4-5 times in beta-thalassemia major and no transfusion was needed in hemoglobin H disease. 3. Postoperatively, there was a significant increase (p less than 0.05) in the liver size by the third year in beta-thalassemia/Hb E disease, and in the first year in beta-thalassemia major but the liver-size was decreased significantly by the fourth year in hemoglobin H disease. 4. Within 2 years postoperatively, the growth velocity in height kept up with their presplenectomy period in hemoglobin H and beta-thalassemia major (except two cases). The growth in weight kept up with their presplenectomy period in 40/49 cases (81.63%) in beta-thalassemia/Hb E but there was no change in the weight velocity in hemoglobin H and beta-thalassemia major. 5. There were 5 cases with immediate postoperative complications. Three cases had pneumonia, one case had septicaemia and one case had bleeding at the operative wound. Episodes of URI decreased in the post-operative period. Five cases of bacteremia developed within 6 years post-splenectomy.(ABSTRACT TRUNCATED AT 250 WORDS)

Wilms' tumor: analysis of thirty cases.

Thirty cases of Wilms' tumor who had been treated at the Ramathibodi Hospital from January 1970 to December 1982 were analysed retrospectively. There were 14 boys and 16 girls, aged 6 months to 7 years (mean age was 2 years). The right kidney was involved in 12 cases, the left side involvement in 18 cases. Other than the abdominal mass, the common signs and symptoms were hematuria (30%) and hypertension (13.3%). The congenital anomalies were found in 2 cases. There was an increase in VMA in three of the six cases determined for VMA:creatine ratio and VMA in 24 hours urine. Seven cases (23.3%) had nephrectomy done in other hospitals. Ninety percent of the patients came in with the stage II-IV, only 10% had stage I. The treatment consisted of surgery, radiation therapy, actinomycin-D, vincristine and adriamycin. Eight patients (26.6%) were lost to follow-up. The cure rate in stage II, III and IV were 71.4%, 50% and 29% respectively. The serious surgical complications include a case of shock due to excessive bleeding and another case of sudden death during the operation due to the tumor emboli from the inferior vena cava to the main pulmonary and both bronchial arteries.

Challenge of the two neural tumors: neuroblastoma and retinoblastoma.

Fifty cases of neuroblastoma and 29 retinoblastoma patients who had additional chemotherapy were analyzed retrospectively. Male:Female ratio were 1:1 and 0.8:1, the ages ranged from birth to 14 years (4 +/- 3.42 year) for neuroblastoma, and one month to 5.5 years (2.4 +/- 1.4 year) for retinoblastoma. More than 70%-90% of them came in advanced stages. The treatment consisted of surgical resection, enucleation or exenteration if feasible. Radiation therapy and chemotherapy were given as specific and palliative measures. All of the neuroblastoma who were younger than 8 months old survived long-term. Various chemotherapeutic agents did not seem to effect the outcome of the advanced cases of these two diseases. The retinoblasts did not seem to be sensitive to MTX and Ara-C. Thio-tepa intrathecally seems to be worth trying. Since these two tumors are not sensitive to treatment and are still a great challenge to the developed countries, awareness of the diseases, early diagnosis and early treatment are appropriate approaches for the developing countries.