Cholesterol ester storage disease: a reported case.

Cholesterol ester storage disease is a rare autosomal recessive disease which is characterized by accumulation of cholesterol esters and triglycerides in the hepatocytes and other tissues. A 5-year and 4-month old Thai female with hepatosplenomegaly and hypercholesterolemia was diagnosed to have this disease by light and electron microscopic studies of the liver tissue from open biopsy. Early diagnosis and treatment with appropriate drug can help the patient by delaying the consequent complications. Genetic counselling and simplified explanation of the disease are a benefit to the patient's family.

Immunophenotype and cytochemical reactions of acute lymphoblastic leukemia in Thai children.

The immunophenotypes of acute lymphoblastic leukemia (ALL) in 28 Thai children were studied by the APAAP technique using a panel of eight specific monoclonal antibodies: HLA-DR, CD 19, CALLA (CD 10), IgM, CD 7, CD 3, CD 4, and CD 8. Sixty-eight, 18, 3.5, 3.5, and 7 per cent were respectively shown to be common, null, pre-B, B, and mature thymocyte T subtypes. Cytochemical reactions (beta-glucuronidase, alpha naphthyl acetate esterase, and acid phosphatase) in this study could identify null, common, and T ALLs with confidence, and could be used in the process of ALL subtyping to reduce cost.

Clinical features of acute lymphoblastic leukemia subclasses in 28 Thai children--a preliminary study.

Twenty-eight Thai children with newly diagnosed acute lymphoblastic leukemia were evaluated for pretreatment characteristic, including immunophenotype of lymphoblast, outcome of treatment, and the correlation among them. By APAAP technique using a panel of eight monoclonal antibodies (HLA-DR, CD 19, CALLA (CD 10), IgM, CD 7, CD 3, CD 4, and CD 8), five subclasses were identified: 67.9, 17.9, 7.1, 3.6, and 3.6 per cent were respectively shown to be common-, null-, mature thymocyte T-, pre B-, and B-ALLs. Clinical features in each subclass conformed to previous reports. All of the 27 evaluable patients attained initial complete remission, but subsequent relapses were noted in 7 patients (25.9%). Three of the 19 cases in the common ALL group relapsed at 6-12 months, whereas, 4 of the 8 cases in the non-common ALL group relapsed at 2-15 months. Probability of relapse at 12 months in the common and non-common ALL groups were 19 and 49 per cent respectively. Disease-free survival from time of remission was shorter in the non-common ALL group. Multivariate analysis of the 6 factors predicting disease-free survival showed that the only strong factor was the immunophenotype of lymphoblast.