Material from 334 consecutive autopsies on Orang Asli subjects performed in the University Hospital, Kuala Lumpur between May 1967 and June 1978 was examined for amyloidosis. Nine positive cases were found, all in patients above 40 years of age, giving an age-corrected incidence of about 9%. In 6 cases, amyloidosis was probably secondary to tuberculosis. The remaining 3 cases exhibited a pericollagenous distribution characteristic of primary amyloidosis. Involvement of the heart and lungs was prominent. However, there were considerable similarities in the distribution and staining properties of the amyloid in the 2 groups. Though both the heart and kidney were frequently affected, the kidney was the most common organ to give rise to clinical symptoms. Infection probably plays a major contributory role in amyloidosis in the Orang Asli.
We report the first documented Malaysian case of aggressive angiomyxoma (AAM) of the vulva. A 56-yr-old woman of Indian ethnic origin presented with a vulval lesion which was clinically mistaken for a Bartholin's cyst. The lesion was surgically excised and a diagnosis of AAM was made histologically. Of particular interest was the finding of foamy and mononuclear inflammatory cells and fibrin in the walls of most of the lesional blood vessels. The patient recovered uneventfully and remains without tumor recurrence at the time of writing 37 mths after initial presentation.
Cancer of the prostate is the sixth most frequently found cancer in Singapore. Prostate-specific antigen (PSA) is the most clinically useful tumour marker available today for the diagnosis and management of prostate cancer. To enhance the value of PSA as a screening test we developed age-specific intervals for our ethnic population. The measurement of free PSA was included in the study to calculate the free:total ratio which enhances the differential diagnosis of prostate cancer from benign prostatic hyperplasia or prostatitis. The total PSA upper limits of 10-year intervals, beginning at 30-years-old, were 1.4, 1.7, 2.3, 4.0, 6.3 and 6.6 microg/l. Free PSA cut-off limits were 0.4, 0.5, 0.5, 1.0, 1.5 and 1.6 microg/l. The free:total ratio of PSA was not age dependent. Abbott AxSym standardised their calibration material for both free and total PSA assays with the Stanford 90:10 reference material. This laboratory has implemented these age-specific reference intervals and are currently following up their pick-up rate in the detection of prostate cancer.
The clinical spectrum of HbH disease varies from a benign disorder to a severe anemia which is blood-transfusion dependent. Heterogeneity at the clinical level is now being understood in terms of the underlying molecular defects. In this study a mild phenotype found in a group of patients with HbH disease is associated with two types of alpha-thalassemia. These are: alpha+-thalassemia (-alpha 3.7/) and alpha 0-thalassemia (--SEA/). In contrast, a second group with more severe HbH disease has a non-deletional alpha-thalassemia defect instead of alpha+-thalassemia (genotype alpha alpha T/--SEA). In the majority of cases, the basis for non-deletional alpha-thalassemia is Hb Constant Spring.
A case of primary hepatocellular carcinoma (PHC) developing in a 10 year old boy who contracted Hepatitis B virus (HBV) infection in the course of maintenance phase chemotherapy for acute lymphoblastic leukemia was seen at University Hospital, Kuala Lumpur. This case is of interest in that it (1) supports an etiological relationship between HBV infection and PHC, (2) manifested a distinctly short malignant transformation time, and (3) draws attention to the possible contributory role of chemotherapy in increasing the risk of developing PHC.