ABSTRACT
Haemoglobin (Hb) Lepore is a variant Hb consisting of two α-globin and two δβ-globin chains. In a heterozygote, it is associated with clinical findings of thalassaemia minor, but interactions with other haemoglobinopathies can lead to various clinical phenotypes and pose diagnostic challenges. We reported a pair of siblings from a Malay family, who presented with pallor and hepatosplenomegaly at the ages of 21 months and 14 months old. The red cell indices and peripheral blood smears of both patients showed features of thalassaemia intermedia. Other laboratory investigations of the patients showed conflicting results. However, laboratory investigation results of the parents had led to a presumptive diagnosis of compound heterozygote Hb Lepore/β-thalassaemia and co-inheritance α+-thalassaemia (-α3.7). Hb Lepore has rarely been detected in Southeast Asian countries, particularly in Malaysia. These two cases highlight the importance of family studies for accurate diagnosis, hence appropriate clinical management and genetic counseling.
ABSTRACT
BACKGROUND: In Malaysia, acute leukemia is the most common cancer among children below the age of 15. A case-control study was here conducted for cases from the Klang Valley, Malaysia, who received treatment at the National University of Malaysia Hospital (HUKM) and Kuala Lumpur General Hospital (GHKL). The main objective was to determine any association with environmental factors. METHODS: Case subjects were children aged below 15 years and diagnosed with acute leukemia in HUKM and GHKL between January 1, 2001 and May 30, 2007. Control subjects were children aged below 15 years who were diagnosed with any non-cancerous acute illnesses in these hospitals. A total of 128 case subjects and 128 control subjects were enrolled in this study. The information was collected using a structured questionnaire and a global positioning system (GPS) device. All factors were analyzed using unmatched logistic regression. RESULTS: The analysis showed that the occurrence of acute leukemia among children was strongly determined by the following factors: family income (odds ratio (OR) 0.19, 95% confidence interval (CI): 0.09-0.42), father with higher social contact (OR 7.61, 95% CI: 3.78-15.4), number of elder siblings (OR 0.36, 95% CI: 0.18-0.77), father who smokes (OR 2.78, 95% CI: 1.49-5.16), and the distance of the house from a power line (OR 2.30, 95% CI: 1.18-4.49). CONCLUSIONS: Some socioeconomic, demographic, and environmental factors are strong predictors of the occurrence of acute leukemia among children in Klang Valley, Malaysia. In terms of environmental factors, it is recommended that future housing areas should be developed at least 200 m away from power lines.