Mixed phenotype acute leukaemia with t(9,22), BCR-ABL1: A case report.

INTRODUCTION: Mixed phenotype acute leukaemia (MPAL) is a rare entity of acute leukaemia. CASE REPORT: Here we report a case of a 39-year-old lady, with an incidental finding of hyperleukocytosis (white blood cells count: 139.2 x 109/L). Her peripheral blood film revealed 36% of blasts and a bone marrow aspiration showed 53% of blasts. Immunophenotyping showed a population of blasts exhibiting positivity of two lineages, myeloid lineage and B-lymphoid lineage with strong positivity of CD34 and terminal deoxynucleotidyl transferase (Tdt). A conventional karyotyping revealed the presence of Philadelphia chromosome. She was diagnosed with MPAL with t(9,22), BCR ABL1, which carried a poor prognosis. She was treated with acute lymphoblastic leukaemia (ALL) chemotherapy protocol coupled with a tyrosine kinase inhibitor and was planned for an allogeneic stem cells transplant. CONCLUSION: This MPAL case was diagnosed incidentally in an asymptomatic patient during medical check-up. We highlight this rare case report to raise the awareness about this rare disease. Understanding the pathogenesis of the disease with the underlying genes responsible for triggering the disease, uniform protocols for diagnosis and targeted treatment will help for proper management of these patients.

Evaluation of coagulation factor activity and sterility of thawed fresh frozen plasma during storage up to 5 days at 4°C.

INTRODUCTION: Fresh frozen plasma (FFP) is a blood component containing functional quantities of all coagulation factors stored at -18°C or below. FFP has to be thawed and transfused as soon as possible to prevent the loss of certain coagulation factor activities and to minimise microbial contamination. MATERIALS AND METHODS: Thirty units of FFP kept at -20°C were thawed using a 37°C water bath and immediately sampled for baseline Factor II (FII), Factor VIII (FVIII) and fibrinogen activity levels and sterility testing. Each unit was then divided into two smaller bags (i.e. Bag I and Bag II) and kept at 4°C. At 6 hours and Day 3, representative samples were taken from Bag I for coagulation factor activity assays, while at Day 5 representative samples were taken from Bag II for coagulation factor activity assays and sterility testing. RESULTS: FII activities at the four time points were 73.43%, 73.73%, 71% and 69.8%, respectively, while FVIII activities were 177.63%, 144.37%, 80.8% and 70.97%, respectively. Fibrinogen levels at the four time points were 3.24 g/L, 3.24 g/L, 3.21 g/L and 3.20 g/L, respectively. All samples were free from microbial contamination even at Day 5. CONCLUSION: The mean reduction in FII and fibrinogen activities on Day 5 was 5% and 1%, respectively. However, FVIII activity declined significantly by approximately 60% at Day 5. Despite these reductions, thawed plasma stored for up to 5 days at 4°C is still suitable for use as the coagulation factor activity levels still exceed the minimum release criteria recommended in quality assurance regulations.

An Audit on Overnight Transfusion Practice in A Teaching Hospital in Malaysia.

INTRODUCTION: Overnight transfusion (OT) is the blood transfusion taking place from 9pm to 8am. During this period, patients are exposed to increased risk of errors. This cross-sectional study aims to determine the incidence and practice of OT in Universiti Kebangsaan Malaysia Medical Centre. MATERIALS & METHODS: Data from all OT in June and mid-July 2017 were collected from recipients' cards, transfusion request forms and patient's case files, regarding discipline involved, indications, time intervals from request of blood transfusion to the completion of OT on patients, monitoring of patients and adverse reactions. RESULTS: A total of 1285 transfusion cases were identified during the study period. 216 (16.8%) cases were OT while the 1069 (83.2%) cases were non-OT. Surgery discipline has the highest (30.1%) OT. The indications of OT were acute clinical need: 82.9%, less acute clinical need: 13.9% and no clinical need: 3.2%. A huge delay (average: 5 hours 40 minutes) in starting transfusion after grouping and crossmatching (GXM) completion was noted. Besides, 25.9% cases took <4 hours to complete OT; 83.4% cases did not have proper transfusion monitoring and three transfusion reactions were reported. DISCUSSION: Although most of the OT cases had appropriate clinical indications, the transfusion can be commenced earlier at day time rather than overnight. Cases without absolute indication should avoid OT. The poor monitoring of patient during OT had posed risks to patients' life if an adverse transfusion reaction happened. The major reason for OTs was a huge delay in starting transfusion after the GXM completion. The contravention of 4-hour infusion rule increased the patients' risk of developing bacterial sepsis. The practice of OT should be discouraged wherever possible except for clinically indicated cases.

Study on ABO and RhD blood grouping: Comparison between conventional tile method and a new solid phase method (InTec Blood Grouping Test Kit).

INTRODUCTION: 'InTec Blood Grouping Test kit' using solid-phase technology is a new method which may be used at outdoor blood donation site or at bed side as an alternative to the conventional tile method in view of its stability at room temperature and fulfilled the criteria as point of care test. This study aimed to compare the efficiency of this solid phase method (InTec Blood Grouping Test Kit) with the conventional tile method in determining the ABO and RhD blood group of healthy donors. METHODS: A total of 760 voluntary donors who attended the Blood Bank, Penang Hospital or offsite blood donation campaigns from April to May 2014 were recruited. The ABO and RhD blood groups were determined by the conventional tile method and the solid phase method, in which the tube method was used as the gold standard. RESULTS: For ABO blood grouping, the tile method has shown 100% concordance results with the gold standard tube method, whereas the solid-phase method only showed concordance result for 754/760 samples (99.2%). Therefore, for ABO grouping, tile method has 100% sensitivity and specificity while the solid phase method has slightly lower sensitivity of 97.7% but both with good specificity of 100%. For RhD grouping, both the tile and solid phase methods have grouped one RhD positive specimen as negative each, thus giving the sensitivity and specificity of 99.9% and 100% for both methods respectively. CONCLUSION: The 'InTec Blood Grouping Test Kit' is suitable for offsite usage because of its simplicity and user friendliness. However, further improvement in adding the internal quality control may increase the test sensitivity and validity of the test results.

Date seed characterisation, substrate extraction and process modelling for the production of polyhydroxybutyrate by Cupriavidus necator.

Poly-3-hydroxybutrate (PHB) is a biodegradable polymer synthesised via bacterial fermentation as a means of storing carbon and energy under unbalanced growth conditions. The production cost of petroleum-based plastics is currently lower than that for biopolymers, and the carbon source is the most significant contributor to biopolymer production cost. A feasibility study to assess the suitability of using a date seed derived media as an alternative for PHB production under various stress conditions was investigated. Results include fructose extraction from date seeds and a mass transfer model to describe the process, demonstrating that the high nutrient content of date seeds makes them a promising raw material for microbial growth and that a meaningful amount of PHB can be produced without supplementation. Maximum dry cell weight and PHB concentrations were 6.3g/l and 4.6g/l respectively, giving a PHB content of 73%, when an initial fructose concentration of 10.8g/l was used.

Lupus podocytopathy: An important differential diagnosis of nephrotic syndrome in systemic lupus erythematosus.

Some patients with systemic lupus erythematosus (SLE) present with sudden onset of nephrotic syndrome and biopsy findings may be of minimal change disease or focal segmental glomerulosclerosis with diffuse foot process effacement on electron microscopy but without significant immune deposits. This entity is termed lupus podocytopathy. Clinicians and renal pathologists need to be aware of this condition. Though steroid sensitive, it needs follow-up to recognize flare and class change, thereby optimizing therapy.

Mechanical unloading of bone in microgravity reduces mesenchymal and hematopoietic stem cell-mediated tissue regeneration.

Mechanical loading of mammalian tissues is a potent promoter of tissue growth and regeneration, whilst unloading in microgravity can cause reduced tissue regeneration, possibly through effects on stem cell tissue progenitors. To test the specific hypothesis that mechanical unloading alters differentiation of bone marrow mesenchymal and hematopoietic stem cell lineages, we studied cellular and molecular aspects of how bone marrow in the mouse proximal femur responds to unloading in microgravity. Trabecular and cortical endosteal bone surfaces in the femoral head underwent significant bone resorption in microgravity, enlarging the marrow cavity. Cells isolated from the femoral head marrow compartment showed significant down-regulation of gene expression markers for early mesenchymal and hematopoietic differentiation, including FUT1(-6.72), CSF2(-3.30), CD90(-3.33), PTPRC(-2.79), and GDF15(-2.45), but not stem cell markers, such as SOX2. At the cellular level, in situ histological analysis revealed decreased megakaryocyte numbers whilst erythrocytes were increased 2.33 fold. Furthermore, erythrocytes displayed elevated fucosylation and clustering adjacent to sinuses forming the marrow-blood barrier, possibly providing a mechanistic basis for explaining spaceflight anemia. Culture of isolated bone marrow cells immediately after microgravity exposure increased the marrow progenitor's potential for mesenchymal differentiation into in-vitro mineralized bone nodules, and hematopoietic differentiation into osteoclasts, suggesting an accumulation of undifferentiated progenitors during exposure to microgravity. These results support the idea that mechanical unloading of mammalian tissues in microgravity is a strong inhibitor of tissue growth and regeneration mechanisms, acting at the level of early mesenchymal and hematopoietic stem cell differentiation.

Hospitalised patients' awareness of their rights: a cross-sectional survey from a tertiary care hospital on the east coast of Peninsular Malaysia.

INTRODUCTION: Optimal patient care varies considerably from place to place and is influenced by scientific as well as social developments. The purpose of this study was to investigate awareness and pertinent issues regarding informed consent among hospitalised patients and to determine lapses, in order to improve the standard of care. METHODS: A questionnaire-based cross-sectional survey was conducted among inpatients at a tertiary care level hospital. RESULTS: 90 percent of patients were aware of their rights, and 85 percent had enough information regarding their illness and modality of treatment. However, treatment options were discussed with 45 percent of cases only, and 65 percent of patients were informed of their duration of treatment. Most of the patients from the surgical group, haemodialysis unit and those with minor ailments were very satisfied with the doctors (92 percent, 86 percent and 96 percent, respectively), as opposed to only 36 percent of cancer patients and 70 percent of acquired immunodeficiency syndrome (AIDS) patients (p-value is less than 0.0001). Almost all patients (99 percent) said that their religious beliefs were respected by the staff and they had no problems in accessing them in times of need. Informed consent was obtained by the doctor in 98 percent of cases and by the nurse in two percent. 98 percent of the patients mentioned that their treatments/examinations were conducted in an atmosphere of privacy and that their personal information was kept confidential by their doctors. CONCLUSION: Patients were reasonably informed about their illness. Their privacy and religious beliefs were duly respected. Treatment options and the duration of treatment were not discussed with all patients. Cancer and AIDS patients were less satisfied with the attending staff. The results suggest that there is a need for periodic surveys of patient satisfaction with the quality of care.

Awareness, knowledge and attitude toward informed consent among doctors in two different cultures in Asia: a cross-sectional comparative study in Malaysia and Kashmir, India.

INTRODUCTION: Informed consent is now accepted as the cornerstone of medical practice, with reasonable patient standards typically considered to be appropriate in the developed countries; however it is still challenged in many developing countries. The objective of this descriptive study was to evaluate the perceptions and practices among attending medical professionals in matters relating to informed consent in selected hospitals. METHODS: A questionnaire-based cross sectional survey among doctors in the two tertiary care hospitals, one in Malaysia and the other in Kashmir, was performed. RESULTS: Awareness on informed consent was universal with "reasonable physician standard" as the most popular choice. As compared to doctors in Malaysia, doctors from Kashmir showed a tendency to reservedly disclose medical information (p-value equals 0.051) and withhold it, if it was deemed potentially harmful (p-value is less than 0.001) or requested so by relatives (p-value is less than 0.023). They also withheld some information from female patients (p-value is less than 0.001). When consent was refused despite needing lifesaving intervention, the majority of both respondents (73 percent versus 80 percent) considered intervention without consent to be justified. Respondents from Malaysia felt that parents could refuse treatment on their children's behalf on the basis of their beliefs (p-value is less than 0.001). CONCLUSION: Despite a very high awareness of informed consent, the model chosen reflected age-old medical paternalism. Doctors' opinions are accorded a larger role in clinical decision-making in Kashmir. The results emphasise the need for doctors to change their attitude and acknowledge the patient's autonomy, which is the basis of modern medical ethics, and yet still be aware of the cultural and religious views of the local population.

Trends in hepatitis B virus infection among blood donors in Kelantan, Malaysia: a retrospective study.

The objective of this study was to determine the prevalence and trends in hepatitis B infection among blood donors attending the Transfusion Medicine Unit at the Hospital Universiti Sains Malaysia, Kelantan, Malaysia. A retrospective study was carried out by reviewing the results of HBsAg among blood donors for the years 2000 to 2004. During this period, 44,658 blood donors were studied. We noted that there was a significant difference in the prevalence of hepatitis B infection between regular and first time donors. There was also a decreasing trend noticed in both study groups. The mean prevalence was significantly different between first time (1.83%) and regular donors (0.45%) (p < 0.005). There is a need to improve public awareness programs to lower the incidence of hepatitis B infection in the general population and consequently first time blood donors. Future studies are also required to determine the trends and outcomes of these programs.

Edwardsiella tarda septicemia with underlying multiple liver abscesses.

Edwardsiella tarda has recently been described as a member of the family Enterobacteriaceae. The genus Edwardsiella contains three species; E. hoshinae, E. ictaluri and E. tarda. Edwardsiella tarda is the only species which has been recognised as pathogenic to humans, especially in those with an underlying disease. The most common presentation is watery diarrhoea. Extra intestinal infections have been reported infrequently. Humans seem to be infected or colonised with Edwardsiella through ingestion or inoculation of a wound. This report is of a patient with multiple liver abscesses due to E. tarda who later developed bacterial peritonitis and septicaemic shock.