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.
Bleeding following bites by the Malayan Pit Viper can either be local or systemic. Bleeding at the site of the bite is due to the local action of the venom as a vasculotoxin. Systemic bleeding occurs with severe poisoning and appears to be mainly dependent on platelet deficiency and the co-existing defibrination syndrome appears to play a minor role in the initiation of bleeding. Thus in the clinical situation non-clotting blood with no overt bleeding can continue up to weeks when specific antivenene is not given. Assessment of the severity of poisoning can easily be made at the bedside. Specific viper antivenene rapidly corrects the spontaneous bleeding and clotting defect of severe systemic poisoning but has no effect on local poisoning.
Systemic lupus erythematosus (SLE) is highly prevalent in Malaysia, which has a mixed population of Malays, Chinese, and Indians. A quantitative enzyme linked immunosorbent assay (ELISA) was used to determine anticardiolipin antibody (aCL) levels (total immunoglobulin, IgG, and IgM) in 200 patients with SLE (164 Chinese, 26 Malay, and 10 Indian) attending the University Hospital of Kuala Lumpur, Malaysia, and 103 matched controls. Only 33 (16.5%) of the patients had raised aCL levels; 26 had raised IgG aCL, five IgM aCL, and two both IgG and IgM aCL. There was a low prevalence of raised levels of aCL in the population studied, which was seen in conjunction with a rare occurrence of thrombosis. The classical association of high aCL levels with thrombocytopenia and recurrent abortions was noted, though not with cerebral disease. The low prevalence of aCL in this study population of mixed racial origin contrasts with findings in European patients with SLE and lends support to the influence of local factors, be they genetic or environmental, on the clinical manifestations of this disease.
From 1981 to 1989, 12 patients of the University Hospital, Kuala Lumpur, were diagnosed to have Evans syndrome based on direct antiglobulin test (DAT) positive haemolytic anaemia and immune thrombocytopenia occurring either simultaneously (7 patients) or consecutively (5 patients). Their mean age at presentation was 24.8 years with a marked female preponderance. All 12 patients were given high dose steroid after diagnosis. Subsequently, other modalities including intravenous immunoglobulin (1 patient) and high dose methylprednisolone (1 patient) were given. Three patients died of intracranial haemorrhage during the first admission while 1 patient died of pulmonary embolism six months after diagnosis. Three patients had splenectomy because of thrombocytopenia. Six patients tested positive for antinuclear factor and antibodies to double stranded DNA and four of them died. Positive serology appeared to be associated with a poorer prognosis. Follow up observations indicate that patients who survive the acute attacks fare reasonably well.
A family demonstrating autosomal dominant thrombocytopenia is described. A 28-year-old Malay housewife was found to have a platelet count of 40 x 10(9)/l with a low mean platelet volume (6.8 fl) while being investigated prior to ovarian cystectomy. The bone marrow was consistent with immune thrombocytopenia but she failed to respond to appropriate therapy. Five siblings, one parent and one nephew have easy bruising and platelet counts of 39-82 x 10(9)/l. Platelet aggregation studies excluded a major functional defect. Survival of homologous platelets in the circulation was normal. Familial thrombocytopenias are rare but important to differentiate from the common acquired thrombocytopenias in order to spare the patient unnecessary treatments.
A retrospective study involving 102 adults with dengue haemorrhagic fever (DHF) was conducted to investigate the demographic aspect, clinical presenting features, laboratory investigations, complications, and mortality associated with the disease. The clinical diagnosis of DHF was in accordance with WHO recommendations. Epistaxis, gingivitis, haematemesis and gastritis were among the common complications. Platelet levels tended to decline from a higher value on admission (mean 67,000/mm3) to lower levels on subsequent days, with the lowest (mean 61,000/mm3) being on day 6 of the fever. Hyponatraemia (46.8%) was commonly observed. Morbidity of DHF was significant (29.4%) but the case fatality rate remained low (2.0%) in our adults, suggesting that adults are less likely than children to suffer from shock syndrome.
Extensive haemangioma with platelet consumption (Kasabach-Merritt syndrome) is rare. The lesion is usually a single cutaneous cavernous haemangioma similar to that found in our patient. Multiple superficial strawberry naevi were also seen all over the rest of the body. This condition was diagnosed antenatally in this patient with the use of colour Doppler. There is one recent report where the thrombocytopaenia was diagnosed by cordocentesis. Unfortunately the baby developed heart failure with intractable coagulopathy and died 2 days later.
Despite concerns about adverse effects, chloramphenicol (CMC) continues to be used in certain situations and, due to its low therapeutic index and variable pharmacokinetics, therapeutic drug monitoring (TDM) is often recommended. At our centre, CMC finds applications in typhoid and meningitis and TDM is routinely performed. Elsewhere in Malaysia, however, CMC is used without TDM. We therefore decided to evaluate our TDM for CMC in relation to its roles in CMC therapy in children, who constitute most of our patients. Our objective was also to develop strategies to improve our TDM for CMC use. Data were collected from 168 children given CMC for various indications and monitored by the TDM service. Plasma CMC was determined by HPLC and used to adjust doses to maintain concentrations within a range of 10-25 micrograms/ml. Outcomes measured included daily temperatures and haematological indices. Daily doses and plasma CMC varied greatly. Doses averaged 40.5 mg/kg for neonates and 75.5 for older children. Average peak concentrations were therapeutic in 60% and trough in 42%. Average duration of fever was 6.3 days and it was unaffected by plasma CMC. Typhoid was eradicated in 97% but nine children with other diagnoses died. Side-effects were confined to mild reversible haematological abnormalities which developed in 11% of children at plasma concentrations which tended to be high. We conclude that CMC remains useful in children with typhoid. Its use for other indications, however, should be reviewed. Routine TDM for CMC is probably not warranted, at least until a clearer role is defined by well designed prospective studies.
Thirty-six patients with lupus nephritis (LN) attending the Nephrology Clinic, Hospital Kuala Lumpur were studied for the prevalence of anticardiolipin antibody (ACA) isotypes (IgG and IgM) and other associated antibodies, antinuclear antibody (ANA) and anti-ds DNA antibody and to determine the possible association between serological and clinical parameters. The study population consisted of 20 (55.6%) Malays, 15 (41.7%) Chinese and 1 (2.8%) Indian with a mean age of 31.4 +/- 11.3 years, range 14 to 60 years. The female to male ratio was 11:1. The average time between diagnosis and blood sampling was 4.4 years (range 0.25 to 15 years). Increased ACA levels were found in 20 (55.6%) patients where raised IgG ACA and IgM ACA were observed in 20 (55.6%) and 2 (5.6%) cases respectively. ANA and anti-ds DNA antibodies were detected in 22 (61.1%) and 4 (11.1%) individuals respectively, with the majority (82%) showing a speckled pattern of nuclear staining. However, neither the IgM ACA nor IgG ACA showed any significant association with thrombosis or any other clinical parametres. Our preliminary study indicates that ACA is a frequent finding in lupus nephritis and that the IgG isotype is more prevalent.
Study site: nephrology Clinic, Hospital Kuala Lumpu
A retrospective analysis of the case records of 494 systemic lupus erythematosus (SLE) patients under follow-up at University Hospital, Kuala Lumpur during 1976-1990 was performed. Overall mortality was 20.2% (100 patients). The causes of death were infection (30%), renal (15%), respiratory (14%), neurological (5%), cardiovascular (7%), other causes (2%) and unknown (27%). Active SLE was a contributing factor in 19% of the deaths. The patients who died had significantly more renal disease, neurological disease, serositis or thrombocytopenia by the end of the first year of disease compared to the survivors. As in other series, infection and active SLE remain important causes of death.
We compared 53 patients with Dengue shock syndrome (DSS) who received preventive transfusions with 53 who did not. Significant differences in the development of pulmonary edema and length of hospitalization (P
This retrospective study analysed the case records of 200 patients in the Department of Gastroenterology, Singapore General Hospital from February 2000 to January 2001 who had liver cirrhosis and underwent gastroscopy for the detection of varices. The aim of this study was to determine any relationship between leucopenia, thrombocytopenia and the occurrence of esophageal varices in a cirrhotic population. Our results showed that the diagnostic yield of varices grade 2 and 3 was 6.3% if platelet count was > 150,000/mm3, 25% if platelet count was 100,000 to 150,000/mm3, 38.9% if platelet count was 50,000-99,000/mm3 and 100% if platelet count was <50,000/mm3. Similarly, the diagnostic yield of varices grade 2 and 3 was 19.4% if total white count was > 4,000/mm3, 66.7% if total white count was 3,000- 4,000/mm3 and 94.8% if total white count was < 3,000/mm5. We conclude that thrombocytopenia and leucopenia can be used to stratify risk for occurrence of esophageal varices in cirrhotic patients and gastroscopy will have a high yield for varices when platelet count is < 150,000/mm3 or total white is < 4,000/mm.
Prospective evaluation of repeated standard tourniquet testing as a diagnostic indicator of dengue infection was done. Included were patients admitted to a children's hospital in Kuala Lumpur on a clinical suspicion of dengue infection based on the World Health Organization (WHO) criteria. A standard method of tourniquet was performed on 79 patients on a daily basis following admission. subjects and negative in the remaining 14 subjects. Fifty-eight subjects were serologically confirmed cases, 4 indeterminate and the remaining 17 subjects had negative serology. For diagnostic classification, 13 had dengue fever, 49 with dengue haemmorhagic fever (DHF) while 17 had non-dengue infection. The sensitivity and specificity of the tourniquet test was 82.8% and 23.5% respectively. The positive predictive value (PPV) was 78.7% while the negative predictive value (NPV) was 28.6%. In addition, the tourniquet test aided in the diagnosis of one-fifth of patients with DHF, who presented with a positive tourniquet test as the only bleeding manifestation. It seems that in a hospital setting, the tourniquet test adds little to the diagnosis of dengue infection/DHF. A positive tourniquet test, repeatedly performed, was found clinically useful as a preliminary screening test in dengue infection as recommended by WHO. However, it was not very specific and had a high false positive rate.
We describe a case of dengue haemorrhagic fever with prolonged thrombocytopaenia. A 22-year-old Malay man with no prior illness presented with a history of fever and generalised macular rash of four days duration. Initial work-up suggested the diagnosis of dengue haemorrhagic fever based on thrombocytopaenia and positive dengue serology. Patient recovered from acute illness by day ten, and was discharged from the hospital with improving platelet count. He was then noted to have declining platelet count on follow-up and required another hospital admission on day 19 of his illness because of declining platelet count. The patient remained hospitalised till day 44 of his illness and managed with repeated platelet transfusion and supportive care till he recovered spontaneously.
BACKGROUND: The current World Health Organisation (WHO) classification of dengue includes two distinct entities: dengue fever (DF) and dengue haemorrhagic fever (DHF)/dengue shock syndrome; it is largely based on pediatric cases in Southeast Asia. Dengue has extended to different tropical areas and older age groups. Variations from the original description of dengue manifestations are being reported.
OBJECTIVES: To analyse the experience of clinicians in using the dengue case classification and identify challenges in applying the criteria in routine clinical practice.
METHOD: Systematic literature review of post-1975 English-language publications on dengue classification.
RESULTS: Thirty-seven papers were reviewed. Several studies had strictly applied all four WHO criteria in DHF cases; however, most clinicians reported difficulties in meeting all four criteria and used a modified classification. The positive tourniquet test representing the minimum requirement of a haemorrhagic manifestation did not distinguish between DHF and DF. In cases of DHF thrombocytopenia was observed in 8.6-96%, plasma leakage in 6-95% and haemorrhagic manifestations in 22-93%. The low sensitivity of classifying DHF could be due to failure to repeat the tests or physical examinations at the appropriate time, early intravenous fluid therapy, and lack of adequate resources in an epidemic situation and perhaps a considerable overlap of clinical manifestations in the different dengue entities.
CONCLUSION: A prospective multi-centre study across dengue endemic regions, age groups and the health care system is required which describes the clinical presentation of dengue including simple laboratory parameters in order to review and if necessary modify the current dengue classification.