We describe an unusual case of osteosarcoma in a Jehovah's Witness patient who underwent chemotherapy and major surgery without the need for blood transfusion. This 16-year-old girl presented with osteosarcoma of the right proximal tibia requiring proximal tibia resection, followed by endoprosthesis replacement. She was successfully treated with neoadjuvant chemotherapy and surgery with the support of haematinics, granulocyte colony-stimulating factor, recombinant erythropoietin and intraoperative normovolaemic haemodilution. This case illustrates the importance of maintaining effective, open communication and exploring acceptable therapeutic alternative in the management of these patients, whilst still respecting their beliefs.
Dengue is endemic in over 100 countries worldwide, predominantly in the subtropical and tropical regions and the incidence has been increasing globally. Patients with severe dengue may develop massive bleeding, disseminated intravascular coagulopathy and multi-organ failure. Bleeding may occur in various body cavities and muscles; however, bleeding in the retroperitoneal space is uncommon. We report a case of a 37-year-old gentleman who presented with a 4-day history of fever associated with chills and rigours. On Day 6 of illness, he complained of left lumbar and left iliac fossa pain which was aggravated by movement. A computed tomography angiography scan of the abdomen showed the presence of a retroperitoneal haematoma, left iliopsoas and quadratus lumbarum intramuscular haematoma with active bleeding and left abdominal wall muscles haematoma. His condition gradually improved after multiple blood transfusions and he gained full recovery. Spontaneous retroperitoneal haematoma is an uncommon complication of severe dengue infection. Early diagnosis based on high index of clinical suspicion using appropriate imaging will aid in prompt management of these cases and may prevent deaths.
T. gondii is a life-threatening infection in immunocompromised patients which may be transmitted through blood transfusion. The present study aimed to evaluate the seroprevalence and molecular detection of T. gondii infection and the associated risk factors among young healthy blood donors in the central part of Mazandaran province, northern Iran. Blood samples were taken from 500 participants and the serum was separated. All serum samples were tested for the presence of anti-T. gondii antibodies (IgG) and then all positive samples were evaluated for IgM antibodies using commercial ELISA kits. All IgM positive samples and 66 randomly selected IgG positive samples were further tested by PCR of the REP-529 gene. Anti-Toxoplasma antibodies (IgG) avidity test was performed for 142 IgG positive samples which were randomly selected. In the current study, anti-T. gondii antibodies (IgG) and (IgM) were found in 316 (63.2%) and 3 (0.95 %) participants, respectively. Seropositivity rate of Toxoplasma was higher among blood donors living in rural areas (P=0.000) and those with a history of soil and animal contact (P<0.05). PCR of the REP-529 gene showed T. gondii DNA in 21 out of 66 samples. The REP-529 gene was not detected in IgM positive samples. Low avidity antibodies (IgG) was found in 23.2% of the IgG positive samples. In conclusions, this study found that the prevalence of toxoplasmosis among young healthy blood donors in north of Iran was high. To reduce the risk of parasite transmission, leukofilteration method are recommended for donated blood used for immunosuppressed patients.
Hereditary haemolytic anaemias have been found to be a significant cause of haemolytic disease in West Malaysia. This paper reports a micromapping study of 916 healthy Malay males from June to August 1983 to determine the distribution of the relevant thalassaemia genes in West Malaysia. Beta thalassaemia trait was found in 2.18%, HbE 3.49% and alpha thal2 (alpha+) trait in 26%. Of the sixteen transfusion dependant Malay thalassaemic patients at the Paediatric Unit, National University of Malaysia, eight patients had HbE beta thalassaemia and the rest are beta thalassaemia major; these patients who are transfusion dependant receive inadequate treatment. Prevention is the only resort.
Transmission of infectious diseases is a recognized complication of blood transfusion and blood products. Nucleic acid testing (NAT) may contribute to improved efficiency of blood screening and thereby increase the safety margin for transfused blood.
Blood group antigen systems are not limited to the ABO blood groups. There is increasing interest in the detection of extended blood group systems on the red cell surface. The conventional method used to determine extended blood group antigens or red cell phenotype is by serological testing, which is based on the detection of visible haemagglutination or the presence of haemolysis. However, this technique has many limitations due to recent exposure to donor red cell, certain drugs or medications or other diseases that may alter the red cell membrane. We aimed to determine the red cell blood group genotype by SNP real time PCR and to compare the results with the conventional serological methods in multiply transfused patients. Sixty-three patients participated in this study whose peripheral blood was collected and blood group phenotype was determined by serological tube method while the genotype was performed using TaqMan®Single Nucleotide Polymorphism (SNP) RT-PCR assays for RHEe, RHCc, Kidd and Duffy blood group systems. Discrepancies were found between the phenotype and genotype results for all blood groups tested. Accurate red blood cell antigen profiling is important for patients requiring multiple transfusions. The SNP RT-PCR platform is a reliable alternative to the conventional method.
H-deficient phenotype individuals with absent or weak anti-H activity may remain undetected on standard routine blood grouping. We report a case of a 59-year-old-man presented with symptomatic anaemia secondary to upper gastrointestinal bleed with haemoglobin level of 68 g/L who required two units of packed red blood cells. He was previously grouped as O Rh D positive and had a history of uneventful multiple blood transfusions. His latest pre-transfusion investigations showed ABO discrepancy between forward and reverse blood grouping, pan-agglutination in both antibody screening and identification with negative direct Coombs test and autocontrol. Further testing including anti-H lectin test and saliva secretor study confirmed that the patient blood group was para-Bombay B RhD positive. This case highlights that the para-Bombay phenotype can be mistakenly labelled as "O" if further investigations are not performed.
1.This paper records the treatment by a continuous intravenous quinine drip technique of fifteen cases of heavy P. falciparum infection in malnourished prisoners of war in a Singapore camp. These cases were selected from a series of approximately 1,000.2.The efficiency of the method, its simplicity, and the ease with which it can be combined with blood transfusion or the slow administration of thiamin are stressed.3.Recovery by this method of treatment is recorded of three cases with a peripheral intensity of infection higher than has hitherto been reported in Malaya with survival.4.The author is of the opinion that this is a safe and effective method for the treatment of pernicious falciparum infections.
Toxoplasmosis is an important parasitic disease in immunosuppressed patients. This prospective study was conducted to determine the seroprevalence, associated risk factors and the incidence of clinically confirmed toxoplasmosis among renal patients at the University of Malaya Medical Center, Kuala Lumpur, Malaysia. We interviewed 247 renal patients, each of whom answered an epidemiological questionnaire, and collected blood samples for measurement of anti-Toxoplasma IgG and IgM antibodies by ELISA. Overall seroprevalence of latent toxoplasmosis was observed in 126 (51%) renal patients. Race (Malays), marital status (married) and primary level of education, were all factors associated with a greater chance of Toxoplasma infection. A case of clinically confirned toxoplasmosis was diagnosed in a renal transplant recipient as a result of immunosuppression. Based on the findings obtained, this preliminary study shows a high prevalence of latent toxoplasmosis in renal patients. Risk factors may have significantly contributed to Toxoplasma acquisition in these patients. We recommend further studies be carried out to monitor for trends in toxoplasmosis among immunosuppressed patients.
This comparative cross-sectional study was conducted in the pediatric daycare unit, Hospital Universiti Sains Malaysia to determine the prevalence of craniofacial deformities (CFD) and the association between these deformities and different clinical presentations among thalassemia patients. Patients were classified as either craniofacial deformity positive (CFD+) or craniofacial deformity negative (CFD-) by two examiners based on the presence or absence of deformity of the cheeks, frontal and/or maxillary bones. Fifteen clinical parameters were compared between the groups. Nineteen out of 43 patients (44.2%; confidence interval, 30.2-58.2%) had craniofacial deformities (CFD+). Both groups were comparable among the clinical parameters studied. Patients in the CFD+ group did not start their blood transfusions significantly earlier than the CFD- group (p = 0.50) and had a nonsignificantly lower mean pretransfusion hemoglobin level than the CFD- group (p = 0.71). Patients receiving regular monthly blood transfusions had a nonsignificantly smaller percentage of CFD than those transfused less often (p = 0.495). CFD+ patients had a splenectomy at a nonsignificantly younger age than CFD- patients (p = 0.36). HbE/beta thalassemia patients were not significantly less likely to develop CFD than other varieties (p = 0.50) and males had a nonsignificantly higher percentage of CFD than females (p = 0.29). This study shows CFD in thalassemia patients are still prevalent but no significant associated factors were found; however, a nonsignificantly higher prevalence of CFD was observed in patients with signs of severe disease and less efficient treatment.
Growth impairment is commonly seen in children with thalassemia despite regular blood transfusions and desferrioxamine treatments. We investigated the growth velocity of 26 prepubertal patients with beta-thalassemia or HbE-beta thalassemia who were transfusion dependent aged between 2 and 13 years. The prevalence of impaired growth velocity (ie, growth velocity less than the third percentile) amongst the transfusion dependent prepubertal thalassemics was 57.7% compared to 19.2% in the control group. The mean height velocity of the thalassemics was 11.1% less than controls but this difference was not statistically significant (4.23cm/year vs 4.76cm/year, p = 0.08). The mean serum ferritin level of the thalassemics with a height < 3rd percentile was higher compared to those with a height > 3rd percentile (4,567.0 vs 2,271.0, p = 0.01). Our study showed that there was a high prevalence of impaired growth velocity amongst our transfusion dependent prepubertal thalassemics. This highlights the problem of inadequate chelation therapy, and compliance with chelation therapy amongst our patients. This study emphasizes the importance of monitoring growth parameters and optimal iron chelation therapy in these patients.
Thalassemia is the commonest hemoglobinopathy in Malaysia. Patients with thalassemia major are transfusion dependent, and a large proportion of them will require splenectomy. As this particular group of patients is immunocompromized, overwhelming sepsis is a recognized complication. We report a series of three patients who all developed intra-abdominal abscesses following splenectomy.
We describe a patient with Evans syndrome (autoimmune hemolytic anemia and autoimmune thrombocytopenia) who was refractory to steroids and intravenous immunoglobulin. She responded to splenectomy and has remained in clinical remission for 3 years. In the majority of cases, splenectomy rarely induces a durable remission but it may be beneficial in a small group of patients, hence should be considered as alternative therapy in the management of these patients.
The aim of this study was to: (1) determine the prevalence and patterns of lung dysfunction among transfusion dependent thalassemics; (2) determine the associated factors that might contribute to this problem. This was a cross-sectional study involving 66 patients with transfusion dependent thalassemia aged 10 years and above. All patients underwent physical examination, standardized pulmonary function tests including spirometry, lung volume, and the carbon monoxide diffusion capacity. A restrictive pattern of lung dysfunction was observed in 22 patients (33.3%) and none showed the presence of obstructive ventilatory impairment. A reduction in the carbon monoxide diffusion capacity (DLCO) was seen 87.9% of the patients, including 7.6% who had evidence of hypoxemia. Ten patients showed a reduction in the FEF25-75% although they did not fulfil the criteria for small airway disease. No correlation was found between lung dysfunction and serum ferritin levels in the patients. Restrictive lung dysfunction and diffusion impairment were the predominant abnormalities found in our cohort of patients.
Study site: Thalassemia clinic, Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM), Kuala Lumpur, Malaysia
One of the major complications in patients with transfusion dependent thalassemia is growth impairment secondary to iron overload. We studied the growth status in 66 patients with beta-thalassemia major and HbE-beta thalassemia who were transfusion dependent, aged from 2 to 24 years, and 66 controls matched for sex and age. The prevalence of short stature in transfusion-dependent thalassemics was 54.5% compared to 4.5% in control group (p<0.001). Short stature was more prevalent in those above the age of 10 years in this study group (83.3% vs 16.7%). Transfusion dependent thalassemics with short stature were found to have significantly lower mean standing height standard deviation scores (SDS), sitting height SDS and subischial leg length SDS values (p<0.001). There was also a significant difference between the mean sitting height SDS and the mean subischial leg length SDS in our thalassemics with short stature, suggesting that the short stature was due to disproportionate truncal shortening. Serum ferritin levels were significantly higher in transfusion dependent thalassemics who were short compared to those who were of normal height (p = 0.002). However, the mean pre-transfusion hemoglobin levels did not differ significantly between patients with short stature and those with normal height (p = 0.216). The prevalence of short stature also did not differ significantly between those with beta-thalassemia major and those with HbE-beta thalassemia (p = 0.32). This study highlighted the importance of providing optimal treatment in these patients, including monitoring of growth parameters and optimizing iron chelation therapy.
Regular blood transfusions for patients with thalassemia have improved their overall survival although these transfusions carry a definite risk of the transmission of certain viruses. Infection with hepatitis B virus (HBV), hepatitis C virus (HCV), cytomegalovirus (CMV) and human immunodeficiency virus (HIV) leads to complications which contribute to the morbidity and mortality of patients with thalassemia. We analyzed the blood samples taken from 85 transfusion dependent thalassemics receiving treatment at the day care center in Hospital Universiti Kebangsaan Malaysia and found that the seroprevalence rates for HBV, HCV and CMV were 2.4%, 22.4% and 91.8% respectively. None of the patients tested positive for HIV. Those positive for HBV and HCV will require further tests and treatment if chronic hepatitis is confirmed.
The aim of this study was to evaluate the management and clinical outcome of transfusion-dependent thalassaemia children receiving care in the Paediatric Ambulatory Care Centre, Hospital Tuanku Ja'afar Seremban in comparison to The Malaysian Clinical Practice Guidelines. The demography, management and clinical outcome of the patients were documented using a checklist. Information on compliance to chelation agents was obtained through interview. There were twenty-six patients recruited in this study out of thirty seven patients registered in the centre. This study showed that more effort and vigilance should be given to ensure that the management of these patients adheres to the guidelines and clinical outcome of these patients monitored closely.
Study site: Paediatric Ambulatory Care Centre, Hospital Tunku Ja'afar, Seremban.