Three cases of myelosclerosis associated with systemic lupus erythematosus are described. The probable role of systemic lupus erythematosus in the initiation of myelonecrosis and subsequent myelosclerosis is discussed.
A case of haemoglobin H (HbH) disease associated with pregnancy is presented and discussed in the light of reports in the literature. The variable symptomatology is commented upon, although mild to moderate chronic haemolytic anaemia seems to be a constant feature. The roles of folic acid supplements and of splenectomy; the avoidance of oxidant drugs, and the mode of inheritance in HbH disease are briefly commented upon. Available reports indicate that HbH disease probably has no adverse effect on pregnancy. However, the association of the two conditions is uncommon, and reports are too few, therefore, to allow definite conclusions on the outcome in all instances.
Two Malaysian Chinese male children are described with Wiskoll-Aldrich syndrome, which has rarely been reported in the East. Classical features were found, with bleeding and infections, low titres of isohaemagglutinins and IgM, but variable IgG and IgA. The immune response was broadly disturbed. Survival of one child was sufficiently long for a Iymphoreticular malignancy or appear.
Five patients illustrate various aspects of obstetrical defibrination in West Malaysia, resulting from exaggeration of changes in fibrinolytic-coagulation equilibrium that occur at delivery. Hypofibrinogenaemia and fibrinolysis may occur in association or either feature predominate. These patients are from a population in which a variety of genetic and environmental factors may interact, e.g. abnormal haemoglobins, cold agglutinins, viral and other infections, introducing additional complications.
A review of acute childhood leukemia in the University Hospital, Kuala Lumpur reveals no significant differences in either the epidemiological or clinical features between Malaysian and Caucasian children. BCG does not appear to have conferred any protection against the occurrence of leukemia. With the introduction of total therapy 4 of 10 patients with good prognostic features and 3 of 15 patients with poor prognostic features have survived 3 years. Prognosis appears to correlate with adopted clinical criteria.
In West Malaysia RA appears to be less common than in temperate climates, but more common than in tropical Africa; furthermore, the incidence of gout and SLE is comparable. The clinical manifestations of RA are milder than those seen in more temperate climates. Subcutaneous rheumatoid nodules have not been observed. Positive serological tests for RF are significantly higher than in the general Malaysian population, but still lower than those reported for patients with RA in temperate climates. Of the three main ethnic groups, the highest incidence of positive results is found in the Chinese.
Study site: Arthritis Clinic, University Hospital, Kuala Lumpur (University Malaya Medical Centre, UMMC, Kuala Lumpur, Malaysia)