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  1. Prevalence and factors associated with vasovagal reaction among whole blood donors in hospital Universiti Sains Malaysia
    Ibrahim NN, Mohd Noor NH, Zulkafli Z
    Transfus Clin Biol, 2023 Jan 23.
    PMID: 36702200 DOI: 10.1016/j.tracli.2023.01.004
    Adverse donor reactions (ADR) are common to occur during the blood donation process. The most common is vasovagal reactions (VVR) and it cause negative impact in the donor return rate. The aim of this study is to determine the prevalence of VVR among blood donors and to study its associated factors, at Hospital University Sains Malaysia (USM). This retrospective case-control study was conducted from June 2018, until June 2021. Data was extracted from the blood bank database system and from donor adverse reaction form. Donors who developed adverse donor reactions were chosen and without VVR were chosen at random as controls. A total of 159 donors, out of 35 134 donors were reported to have VVR which resulted in an overall prevalence of 0.45 %. Dizziness or mild VVR were the most frequently observed adverse reactions, accounting for approximately 87/159 (54.7 %) of all adverse reactions. Multiple logistic regression (MLR) analysis showed VVR were significantly associated with age, female gender, first-time donor, and 450 ml volume of blood collected. The prevalence of vasovagal reactions among blood donors in this study was low which was similar to a few previous studies. Although it was low, still it is very important to reduce risks to a minimum so that the donor return rate could be maintained. The information regarding its associated factors can be used to identify high-risk donors to prevent the incidence in the future.
  2. Fulltext Hemolytic disease of fetus and newborn due to maternal red blood cell alloantibodies in the Malay population
    Hassan MN, Mohd Noor NH, Johan Noor SR, Sukri SA, Mustafa R, Luc Aster HV
    Asian J Transfus Sci, 2014 Jul;8(2):113-7.
    PMID: 25161351 DOI: 10.4103/0973-6247.137449
    Maternal red blood cell (RBC) alloimmunization may lead to production of harmful antibodies that result in hemolytic disease of fetus and newborn (HDFN). There is insufficient data on the prevalence of HDFN due to RBC alloantibodies in the Malay neonatal population.
  3. Severe anti-D haemolytic disease of fetal and newborn in rhesus D negative primigravida
    Hassan MZ, Iberahim S, Abdul Rahman WSW, Zulkafli Z, Bahar R, Ramli M, et al.
    Malays J Pathol, 2019 Apr;41(1):55-58.
    PMID: 31025639
    INTRODUCTION: Anti-D alloimmunisation may occur from the blood transfusion or fetomaternal haemorrhage which can lead to haemolytic disease of fetal and newborn (HDFN). The morbidity and mortality of HDFN related to anti-D is significantly reduced after introduction of anti-D prophylaxis and furthermore, anti-D HDFN in RhD negative primigravida is uncommonly seen.

    CASE REPORT: A case of unusual severe HDFN due to anti-D alloimmunisation in undiagnosed RhD negative primigravida Malay woman is reported here. This case illustrates the possibility of an anamnestic response from previous unknown sensitisation event or the development of anti-D in mid trimester. The newborn expired due to hydrops fetalis and severe anaemia. Antenatally, the mother was identified as RhD positive and thus there was no antenatal antibody screening, antepartum anti-D prophylaxis or close fetal monitoring for HDFN.

    DISCUSSION: The thorough antenatal ABO and RhD blood grouping with antibody screening is mandatory as part of prevention and early detection of HDFN especially due to anti-D alloimmunisation. Improper management of RhD negative women might lead to severe HDFN including in primigravida.

  4. Fulltext Molecular Detection of Alpha Thalassemia: A Review of Prevalent Techniques
    Vijian D, Wan Ab Rahman WS, Ponnuraj KT, Zulkafli Z, Mohd Noor NH
    Medeni Med J, 2021;36(3):257-269.
    PMID: 34915685 DOI: 10.5222/MMJ.2021.14603
    Alpha thalassemia (α-thalassemia) is an autosomal recessive disorder due to the reduction or absence of α globin chain production. Laboratory diagnosis of α-thalassemia requires molecular analysis for the confirmatory diagnosis. A screening test, comprising complete blood count, blood smear and hemoglobin quantification by high performance liquid chromatography and capillary electrophoresis, may not possibly detect all the thalassemia diseases. This review focused on the molecular techniques used to detect α-thalassemia, and the advantages and disadvantages of each technique were highlighted. Multiplex gap-polymerase chain reaction, single-tube multiplex polymerase chain reaction, multiplex ligation-dependent probe amplification, and loop-mediated isothermal amplification were used to detect common deletion of α-thalassemia. Furthermore, the reverse dot blot analysis and a single tube multiplex polymerase chain reaction could detect non-deletion mutation of the α-globin gene. Sanger sequencing is widely used to detect non-deletion mutations of α-thalassemia. Recently, next-generation sequencing was introduced in the diagnosis of both deletion and point mutations of α-thalassemia. Despite the advantages and disadvantages of different techniques, the routine method employed in the laboratory should be based on the facility, expertise, available equipment, and economic conditions.
  5. Fulltext Red Blood Cell Alloimmunization and Its Associated Factors among Chronic Liver Disease Patients in a Teaching Hospital in Northeastern Malaysia
    S Abdullah SZ, Hassan MN, Ramli M, Abdullah M, Mohd Noor NH
    Diagnostics (Basel), 2023 Feb 25;13(5).
    PMID: 36900030 DOI: 10.3390/diagnostics13050886
    Red blood cell (RBC) alloimmunization is an important complication of blood transfusion. Variations in the frequency of alloimmunization have been noted among different patient populations. We aimed to determine the prevalence of RBC alloimmunization and associated factors among chronic liver disease (CLD) patients in our center. This is a case-control study involving 441 patients with CLD who were being treated at Hospital Universiti Sains Malaysia and subjected to pre-transfusion testing from April 2012 until April 2022. Clinical and laboratory data were retrieved and statistically analyzed. A total of 441 CLD patients were included in our study, with the majority being elderly, with the mean age of patients 57.9 (SD ± 12.1) years old, male (65.1%) and Malays (92.1%). The most common causes of CLD in our center are viral hepatitis (62.1%) and metabolic liver disease (25.4%). Twenty-four patients were reported to have RBC alloimmunization, resulting in an overall prevalence of 5.4%. Higher rates of alloimmunization were seen in females (7.1%) and patients with autoimmune hepatitis (11.1%). Most patients developed a single alloantibody (83.3%). The most common alloantibody identified belonged to the Rh blood group, anti-E (35.7%) and anti-c (14.3%), followed by the MNS blood group, anti-Mia (17.9%). There was no significant factor association of RBC alloimmunization among CLD patients identified. Our center has a low prevalence of RBC alloimmunization among CLD patients. However, the majority of them developed clinically significant RBC alloantibodies, mostly from the Rh blood group. Therefore, phenotype matching for Rh blood groups should be provided for CLD patients requiring blood transfusions in our center to prevent RBC alloimmunization.
  6. Fulltext Hereditary Protein S Deficiency With an Extensive Femoral Artery Thrombosis
    Mohd Noor NH, Che Anuar NA, Saidin NIS, Iberahim S, Abdullah AH
    Cureus, 2023 Dec;15(12):e51355.
    PMID: 38292964 DOI: 10.7759/cureus.51355
    Protein S (PS) deficiency is widely recognized for its connection to venous thromboembolism risk. However, the relation between PS deficiency and arterial thrombotic events (ATEs) remains uncertain. Here, we report a patient who experienced an ATE with a family history of PS deficiency. We highlight an attention to the issues related to the management of arterial thrombotic events and discuss the potential use of antiplatelet therapy as a treatment option for a specific group of patients diagnosed with PS deficiency.
  7. Fulltext Mannose-binding lectin gene sequence data in Kelantan population
    Zahidin MA, Mohd Noor NH, Johan MF, Abdullah AD, Zulkafli Z, Edinur HA
    Sci Data, 2024 Apr 30;11(1):435.
    PMID: 38688916 DOI: 10.1038/s41597-024-03274-4
    The human mannose-binding lectin (MBL) gene encodes a polymorphic protein that plays a crucial role in the innate immune response. Human MBL deficiency is associated with immunodeficiencies, and its variants have been linked to autoimmune and infectious diseases. Despite this significance, gene studies concerning MBL sequencing are uncommon in Malaysia. Therefore, we aimed to preliminary described the human MBL sequencing dataset based on the Kelantan population. Blood samples were collected from 30 unrelated individuals and underwent DNA extraction, genotyping, and sequencing. The sequencing data generated 886 bp, which were deposited in GenBank (ON619541-ON619546). Allelic variants were identified and translated into six MBL haplotypes: HYPA, HYPB, LYPB, LXPB, HXPA, and LXPA. An evolutionary tree was constructed using the haplotype sequences. These findings contribute to the expansion of MBL information within the country, providing a valuable baseline for future research exploring the association between the gene and targeted diseases.
  8. Fulltext Clinical and Laboratory Diagnosis of Antiphospholipid Syndrome: A Review
    Zahidin MA, Iberahim S, Hassan MN, Zulkafli Z, Mohd Noor NH
    Cureus, 2024 Jun;16(6):e61713.
    PMID: 38975541 DOI: 10.7759/cureus.61713
    The antiphospholipid syndrome (APS) manifests through venous or arterial thrombosis, with or without pregnancy complication alongside the continuous presence of antiphospholipid antibodies (aPL). APS classification relies on three aPL subtypes: anticardiolipin (aCL), anti-β2-glycoprotein I antibodies (anti-β2GPI), and lupus anticoagulants (LA) antibodies. Given that thrombosis and pregnancy issues are not unique to APS, the precise and reliable identification of aPL forms the basis for diagnosis. Semi-quantitative solid-phase assays identify two antibodies, aCL and anti-β2GPI, while LA detection occurs through various phospholipid-dependent coagulation assays that are based on antibody behaviour. LA, specifically, is conclusively associated with thrombosis, prompting discussions around the serological criteria for APS. Despite advancements in LA detection, the standardisation of all aPL detection assays remains imperative. The combined presence of aCL and anti-β2GPI with thrombosis inconsistently triggers concern. Initial presentations by APS patients commonly exhibit a heightened risk of stroke, miscarriages in the later stages of pregnancy, positive results of LA tests, and widespread thrombosis across multiple organs, often leading to adverse outcomes. Correctly diagnosing this condition is pivotal to avoid unnecessary long-term secondary thromboprophylaxis.
  9. Fulltext Bacterial Cellulose: Production, Characterization, and Application as Antimicrobial Agent
    Lahiri D, Nag M, Dutta B, Dey A, Sarkar T, Pati S, et al.
    Int J Mol Sci, 2021 Nov 30;22(23).
    PMID: 34884787 DOI: 10.3390/ijms222312984
    Bacterial cellulose (BC) is recognized as a multifaceted, versatile biomaterial with abundant applications. Groups of microorganisms such as bacteria are accountable for BC synthesis through static or agitated fermentation processes in the presence of competent media. In comparison to static cultivation, agitated cultivation provides the maximum yield of the BC. A pure cellulose BC can positively interact with hydrophilic or hydrophobic biopolymers while being used in the biomedical domain. From the last two decades, the reinforcement of biopolymer-based biocomposites and its applicability with BC have increased in the research field. The harmony of hydrophobic biopolymers can be reduced due to the high moisture content of BC in comparison to hydrophilic biopolymers. Mechanical properties are the important parameters not only in producing green composite but also in dealing with tissue engineering, medical implants, and biofilm. The wide requisition of BC in medical as well as industrial fields has warranted the scaling up of the production of BC with added economy. This review provides a detailed overview of the production and properties of BC and several parameters affecting the production of BC and its biocomposites, elucidating their antimicrobial and antibiofilm efficacy with an insight to highlight their therapeutic potential.
  10. Sagittal sinus thrombosis in a patient with familial Protein C deficiency: Highlighting the impact of thrombophilia testing
    Wan Ab Rahman WS, Abdullah WZ, Hassan MN, Ahmed S, Zulkafli Z, Wan Ahmed WA, et al.
    Malays J Pathol, 2021 Dec;43(3):449-452.
    PMID: 34958066
    Plasma protein-C is a natural anticoagulant that inactivates factors Va and VIIIa. Familial protein C deficiency is inherited as an autosomal dominant disorder. The homozygous or compound heterozygous type may present early as purpura fulminant, while the heterozygous type can present as thromboembolism later in life. Presented in this report is a case of a 21-year-old female patient with protein-C deficiency, confirmed by thrombophilia investigations. She experienced recurrent deep vein thrombosis and cerebral sinus thrombosis due to thrombotic occlusion. She had a family history of deep vein thrombosis. Hence, high-risk cases should be seriously considered for long term anticoagulation therapy. The utility versus futility of thrombophilia testing in a particular situation is discussed to address and ensure safe practice among patients with thromboembolism.
  11. Platelet Additive Solutions as an Alternative Storage Medium of Apheresis Platelets to Reduce ABO Antibody Titer for ABO-Incompatibility Platelet Transfusion
    Adzahar S, Hassan MN, Zulkafli Z, Mohd Noor NH, Ramli M, Mohamed Yusoff S, et al.
    Cureus, 2023 Aug;15(8):e44012.
    PMID: 37746385 DOI: 10.7759/cureus.44012
    Introduction Platelet additive solutions (PASs) are nutrient media commonly used to replace and reduce the need for storage plasma. They are an alternative medium to maintain high-quality platelets lasting longer on the shelf for about seven days. Platelets with high titer of ABO antibody can pose a hemolytic transfusion reaction (HTR) risk if units are given across the ABO barrier. The risk of complication is greater when group O platelet is released to non-group O patients. The PAS has been known as a safe medium, where the titer of ABO antibodies is expected to be diluted. In this study, we compared the anti-A and anti-B antibody titers of apheresis platelets in PAS and non-PAS (plasma) as the suspending media. Methods A total of 20 apheresis platelet donors were selected, with seven from blood group A, eight from blood group B, and five from blood group O. The platelets were collected using an Amicus cell separator. They were suspended in PAS and plasma before being stored at a temperature range of 22-24º C. Anti-A (blood group B and O) and Anti-B (blood group A and O) antibody titers were measured and compared between the two suspending media. Wilcoxon signed-rank test is used for statistical analysis, and a p-value <0.05 is considered significant. Results The median titer of the anti-A antibody of apheresis platelets showed a significant difference between suspended in PAS (2.50) and plasma (4.00), p=0.002. Similar findings were also seen with the median titer of the anti-B antibody of apheresis platelet, in which it showed a significant difference between suspended in PAS (2.00) and plasma (4.00), p=0.004. It was observed that there was a significant reduction in both anti-A and anti-B antibody titers in the PAS as compared to the plasma group. Conclusion The decrease in ABO antibody titer in apheresis platelets stored with PAS can be beneficial for patients. This reduces the risk of HTRs if ABO-incompatible platelet units need to be issued. Thus, using PAS as a storage medium significantly improves platelet inventory management without compromising patient safety.
  12. An Epidemiology Study of Adult Immune Thrombocytopenia Patients in a Teaching Hospital in Northeastern Malaysia
    Zahidin MA, Abdul Razak NI, Mohd Noor NH, Johan MF, Zulkafli Z, Abdullah AD, et al.
    Cureus, 2023 Nov;15(11):e48533.
    PMID: 38073971 DOI: 10.7759/cureus.48533
    Immune thrombocytopenia (ITP) has been comprehensively studied and understood in Western Europe and various Asia-Pacific regions. However, the epidemiological and clinical-laboratory aspects of ITP in Malaysia remain limited and not well documented. Therefore, this study aims to evaluate the incidence and clinical parameters of ITP using 20-year retrospective data. Medical records for 205 consecutive adult patients with ITP between January 2000 and December 2022 were analyzed. A p-value of <0.05 is considered statistically significant. The majority were Malays (n=192, 93.7%) and females (n=150, 73.2%), with a male-to-female ratio of 1:2.73. One hundred thirty-two (64.4%) and 73 (35.6%) patients were diagnosed with primary ITP (pITP) and secondary ITP (sITP), respectively. Systemic lupus erythematosus (SLE) (n=23, 35.9%), antiphospholipid syndrome (APS) (n=5, 7.8%), and familial thrombocytopenia (n=5, 7.8%) were the top 3 comorbid conditions for ITP. The overall incidence was 1.80/100,000 person-years (95% confidence interval (CI): 1.56-2.07), and the incidences were higher in females than in males with pITP (1.78/100,000 person-years versus 0.70/100,000 person-years) and sITP (0.86/100,000 person-years versus 0.26/100,000 person-years). The median age for patients with pITP was significantly higher than for those with sITP (median: 44 versus 37 years, respectively) (p=0.026). However, there was no statistically significant difference in white blood cell (WBC) counts, hemoglobin (HB) counts, platelet (PLT) counts, absolute neutrophil counts (ANC), or hematocrit (HCT) counts between those with pITP and sITP at the time of diagnosis. The current study provides an overview of ITP epidemiology in northeastern Malaysia. We emphasize the critical need for further additional research, particularly at the state and national levels in the future.
  13. Fulltext Haemostatic, Inflammatory, and Haematological Biomarkers Among Orthopaedic Patients With Prolonged Immobilization and the Risk of Hypercoagulable States
    Ramli NN, Iberahim S, Mohd Noor NH, Zulkafli Z, Tengku Shihabuddin TM, Din MH, et al.
    Cureus, 2024 Jan;16(1):e51552.
    PMID: 38313946 DOI: 10.7759/cureus.51552
    Background Venous thromboembolism (VTE) is a common and potentially life-threatening complication in patients with lower limb traumatic fractures. Orthopaedic patients who experience trauma in the lower limbs with prolonged immobilization may experience a hypercoagulable state, which could eventually lead to the development of VTE. Therefore, this study aims to evaluate the changes in hypercoagulable markers, including haemostatic, inflammatory, and haematological biomarkers in orthopaedic trauma patients with prolonged immobilization. Materials/method This prospective cohort study was conducted at Hospital Universiti Sains Malaysia from August 2020 to March 2022. Every patient with fractures in the lower limbs was screened for eligibility, and patients who required immobilization for more than five days without receiving anticoagulant prophylaxis were recruited for this study. The laboratory tests, including D-dimer, fibrinogen, prothrombin time (PT), activated partial thromboplastin time (aPTT), erythrocyte sedimentation rate (ESR), and platelet count, were serially measured on day one and day five of hospitalization. The biomarkers were analyzed using a paired t-test, with a p-value <0.05 as a significant result. Results A total of 54 patients with fractures in the lower limbs, ages ranging from 12 to 50 years old, were involved in this study. The paired t-test analysis demonstrated that several biomarkers showed a significant increase in mean difference between day one and day five of immobilization, which included fibrinogen, ESR, and platelet count. The mean differences for each biomarker with fibrinogen were 0.66 g/L (p<0.001, 95% CI of mean difference: -1.04, -0.27), ESR increased by 17.98mm/hr (p<0.001, 95% CI of mean difference: -24.69, -11.27), and platelet count increased by 128.59×109/L (p<0.001, 95% CI of mean difference: -166.55, -90.64) on day five of immobilization. D-dimer was elevated in all patients on both post-trauma days; however, no significant difference was observed in this biomarker between day one and day five of immobilization. Conclusion In conclusion, our study found that fibrinogen, ESR, and platelet count levels were significantly increased in orthopaedic trauma patients with prolonged immobilization. The increase in these biomarkers indicates the body's reaction to tissue injury after trauma, which may contribute to the hypercoagulable states. Further research with a larger sample size is warranted to assess the viability of these biomarkers as potential diagnostic indicators for the development of VTE related to hypercoagulability.
  14. The Blood Donor Deferral Rate and the Reasons for Deferral at a Tertiary Care Teaching Institute in Northeastern Malaysia
    Zahidin MA, Saidin NIS, Ibrahim NA, Mohd Nasir NNA, Abdul Razak NI, Ramli M, et al.
    Cureus, 2024 Feb;16(2):e54954.
    PMID: 38544652 DOI: 10.7759/cureus.54954
    A deferral takes place when donors fail to meet the eligibility criteria for donating blood during their visit to a blood collection site. Deferral periods, which can be either permanent or temporary, are implemented to protect the well-being of both the donor and the recipient. This study aimed to investigate the frequency of deferrals and the various factors contributing to them. A retrospective analysis was conducted at the Transfusion Medicine Unit of Hospital Universiti Sains Malaysia (USM), utilizing data obtained from blood donors during the period from January 2022 to June 2023. The research included a cohort of 18,751 donors who visited our transfusion unit for blood donation. Data, including gender, age, and reasons for deferral, were collected by reviewing the records of donors who were deferred. Descriptive statistics were employed to analyze the data of deferral blood donors. Out of 18,751 blood donors, 3,533 (18.84%) were deferred, consisting of 1,267 males (35.86%) and 2,266 females (64.14%). The age group of 18-25 years accounted for the highest number, comprising 1,875 donors (53.07%). Among the deferred cases, 53.33% were first-time donors, followed by 25.28% regular donors and 21.40% lapsed donors. The deferral of blood donors resulted from various reasons. The most common cause of overall deferral among blood donors was low hemoglobin (38.33%), followed by upper respiratory tract infections (8.38%), chronic medical illness (7.08%), and high blood pressure (7.02%). Temporary deferrals were more prevalent than permanent deferrals, accounting for 91.57% of cases compared to 8.43% for permanent deferrals. Voluntary non-remunerative blood donors constitute the backbone for a safe and reliable blood supply in transfusion services. Utilizing a comprehensive database will enable effective counseling of temporarily deferred donors, providing insights into the reasons for their deferral, the expected duration, and the appropriate treatments. This information is crucial for motivating these donors to recruit again in the donor pool. Public education initiatives aimed at raising awareness about the causes of deferral and promoting regular health check-ups can play a pivotal role in minimizing these deferrals.
  15. A rare case of compound heterozygous Southeast Asian double α-globin gene deletion and Haemoglobin Quong Sze in a Malay proband
    Vijian D, Wan Ab Rahman WS, Kannan TP, Zulkafli Z, Mohd Noor NH, Bahar R, et al.
    Malays J Pathol, 2024 Aug;46(2):321-324.
    PMID: 39207010
    INTRODUCTION: Haemoglobin (Hb) Quong Sze is a non-deletional α-thalassaemia subtype that occurs due to missense mutation at codon 125 of the HBA2 gene. Interaction between Hb QS with Southeast Asian double α-globin gene deletion results in non-deletional HbH disease, which is more severe than deletional HbH.

    CASE REPORT: A 3-month-old baby boy was presented with neonatal anaemia and mild hepatomegaly. Full blood count revealed severe hypochromic microcytic anaemia. There was an abundance of HbH inclusion bodies in his red blood cells. High-performance liquid chromatography showed a reduced HbA2 level with the presence of pre-run peak. Capillary electrophoresis showed the presence of HbH and Hb Barts. Molecular analysis found a common α0-thalassaemia (--SEA) in one allele and mutation in codon 125 in the other allele.

    DISCUSSION: Non-deletional HbH disease due to a combination of deletional and non-deletional mutations may present with severe clinical manifestations than those with deletion mutations, which warrants accurate diagnosis using molecular techniques.

  16. Fulltext Blood Transfusion Knowledge among Nurses in Malaysia: A University Hospital Experience
    Mohd Noor NH, Saad NH, Khan M, Hassan MN, Ramli M, Bahar R, et al.
    Int J Environ Res Public Health, 2021 10 25;18(21).
    PMID: 34769712 DOI: 10.3390/ijerph182111194
    Blood transfusion is a fundamental and life-saving procedure where the consequence of errors can be fatal. Nurses' knowledge plays an essential role in ensuring quality and safety in blood transfusion. The objective of this study was to assess blood transfusion-associated knowledge of tertiary hospital nurses on the east coast of Malaysia. This was a cross-sectional study with 200 registered nurses involved in blood transfusion procedures at Hospital Universiti Sains Malaysia. The knowledge of the nurses was evaluated by using the routine blood transfusion knowledge questionnaire based on five parts, and <50%, 50-74%, or ≥75% of the knowledge was considered as poor, moderate, or high, respectively. Based on the scoring system, the overall knowledge of blood transfusion among Malaysian nurses (33.2 ± 8.4 years) was estimated to be 54.9 ± 7.6%. In individual items, the scoring was 81.0%, 45.4%, 49.2%, 63.0%, and 90.0% in knowledge prior to blood transfusion, on pre-transfusion, on post-transfusion, on complications, and on transfusion policy, respectively. The findings of this study indicated that most of the nurses' overall knowledge of blood transfusion was at a moderate level; therefore, training courses and continuous medical education are warranted to improve knowledge and skills of the nurses to ensure good practices of blood transfusion.
  17. Acute hemolytic transfusion reaction following ABO-mismatched platelet transfusion: Two case reports
    Zulkeflee RH, Hassan MN, Hassan R, Saidin NIS, Zulkafli Z, Ramli M, et al.
    Transfus Apher Sci, 2023 Jun;62(3):103658.
    PMID: 36805153 DOI: 10.1016/j.transci.2023.103658
    Acute hemolytic transfusion reaction following ABO-incompatible platelet transfusion: two case reports An ideal platelet transfusion should provide ABO identical platelet concentrate, and cross match compatibility is not routinely performed in the standard practices. However, ABO non identical platelet transfusions are not uncommon with the limited resources and short shelf life of platelet concentrate. Though rare, acute hemolytic transfusion reaction (AHTR) may occur following minor ABO-incompatible platelet transfusion. Here, we report two cases of thrombocytopenic patients (one child and one adult) type as Group B RhD positive and received Group O RhD positive platelet transfusions. Both patients experienced an AHTR evidenced by a drop in hemoglobin level, spherocytosis and small agglutinations on the blood film, and positive direct Coombs test. They were treated symptomatically, recovered and discharged well post-event without any morbidity. No anti-B isohemagglutinins titer were done to confirm the high titer of the antibody in the platelet donors. Our cases highlighted the importance of ABO-compatible platelet transfusion, especially to children and those vigilant groups of patients.
  18. Fulltext Significance of Zone 2 Peak on Capillary Electrophoresis in the Detection of Hemoglobin Constant Spring
    Ramli M, Nik Mohd Hasan NFF, Ramli M, Wan Ab Rahman WS, Hassan MN, Mohd Noor NH, et al.
    Oman Med J, 2023 May;38(3):e507.
    PMID: 37351377 DOI: 10.5001/omj.2023.78
    OBJECTIVES: Hemoglobin constant spring (Hb CS) is a point mutational defect associated with α thalassemia. The aims of this study were to compare the hematological profiles between different Hb CS genotypes and to estimate the range for Zone 2 peak using capillary electrophoresis (CE) with different Hb CS genotypes.

    METHODS: For this cross-sectional study, patient blood samples that showed a positive peak in zone 2 of CE were selected. Hemoglobin and DNA of the samples were investigated to ascertain the presence and levels of non-deletional and deletional α thalassemia. The results were statistically analyzed.

    RESULTS: Of the 137 samples investigated, 118 (86.1%) were positive for termination codon Hb CS mutation. Heterozygous Hb CS was found in 92 (67.2%), compound heterozygous Hb CS in 22 (16.1%), and homozygous Hb CS in four (2.9%) samples. The ranges of Hb CS level for heterozygous Hb CS, compound heterozygous Hb CS, and homozygous Hb CS were within 0.2-2.7%, 0.3-2.2%, and 4.5-5.5%, respectively. Significant hematological differences in the Hb level, mean cell volume, mean cell hemoglobin, red cell distribution width, red blood cell count, and Hb CS level were observed between heterozygous, homozygous, and compound heterozygous Hb CS.

    CONCLUSIONS: In view of the overlapping prevalence range of Hb CS level for heterozygous and compound heterozygous Hb CS, only Hb CS level within the range 4.5-5.5% was helpful in the diagnosis of homozygous Hb CS.

  19. Fulltext Coagulation Status Using Clot Wave Analysis in Patients With Prolonged Immobilization
    Iberahim S, Muhamat Yusoff R, Mohd Noor NH, Hassan R, Ramli NN, Bahar R, et al.
    Cureus, 2024 Jan;16(1):e51483.
    PMID: 38304638 DOI: 10.7759/cureus.51483
    Background Prolonged immobilization is widely recognized as a risk factor for thromboembolism. In this prospective study, we investigated the changes in clot waveform analysis (CWA) parameters in prolonged immobilized patients following lower limb trauma. CWA is an advanced method for assessing global coagulation that involves continuously monitoring changes in light transmittance, absorbance, or light scattering during routine clotting tests. Additionally, we also aim to determine the CWA parameters between day one and after day three of immobilization. Methods A total of 30 patients with prolonged immobilization were enrolled in this study. The plasma of these patients was collected on the first day of their admission and subsequently obtained again after day three of immobilization. Prothrombin time (PT)-based CWA and activated partial thromboplastin time (aPTT)-based CWA were performed using the ACL TOP 300 CTS (Werfen: Bedford, USA) coagulation analyzer, which utilizes the optical method for clot detection. Plasma samples for 20 normal controls were recruited from a healthy blood donor. The CWA parameters generated during clot formation were analyzed. For the comparison of CWA parameters between patients with prolonged immobilization and healthy controls, the Mann-Whitney test was used. A paired t-test was used for the comparison of clot wave parameters between day one and after day three of immobilization. This study was approved by the Universiti Sains Malaysia Research Ethics Committee. Result The mean values of PT and aPTT in healthy controls were 11.66 seconds and 33.98 seconds, respectively. There was no statistically significant difference between the patients and the healthy controls in the median values of aPTT (P=0.935). However, patients with prolonged immobilization exhibited significantly higher median PT CWA parameter values than controls (P=0.007). These parameters included the delta change (P<0.001), peak time velocity (P=0.008), and height velocity (P<0.001). On the other hand, the delta change (P<0.001) and height velocity (P<0.001) of the aPTT CWA parameters were significantly higher in patients with prolonged immobilization than in controls. In patients with prolonged immobilization, there was no significant difference in PT CWA parameters between day one and after day three of immobilization, while for aPTT CWA, all parameters were higher on day three, except for the endpoint time. Conclusion Patients with prolonged immobilization exhibit increased PT and aPTT CWA parameters compared to normal controls. CWA parameters could aid in identifying patients at risk of developing thrombosis through changes in the clot waveform. However, further study is needed to fully utilize additional information from routine coagulation testing.
  20. Hematological Profile of Hb Adana Among High School Students in Northeast Peninsular Malaysia
    Siti Asmaa MJ, Miin Phoon L, Zakaria NA, Hussin S, Bahar R, Hassan MN, et al.
    Cureus, 2024 Mar;16(3):e57353.
    PMID: 38694420 DOI: 10.7759/cureus.57353
    Background Hb Adana is a non-deletional alpha (α)-thalassaemia variant resulting from mutations in α1- or α2-globin codon 59 (αCD59), leading to the production of unstable α-globin. Clinical manifestations can vary from silent carrier status to dependence on blood transfusions, hepatosplenomegaly, skeletal deformities, and spinal cord compression. Despite the significance of Hb Adana inheritance, studying this variant poses challenges due to the scarcity of molecular tests and the potential for routine diagnoses to be overlooked. This study aims to investigate the prevalence of Hb Adana among local high school students and assess the hematological parameters and hemoglobin analysis of Hb Adana in Malaysia. Methodology This retrospective study analyzed 13,721 blood samples collected from high school students participating in Malaysia's National Thalassaemia Screening Program at Hospital Raja Perempuan Zainab II (HRPZ II). Deletional α-thalassaemia was detected using multiplex gap-polymerase chain reaction (PCR), while common non-deletional α-thalassaemia was identified using multiplex amplification refractory mutation system (ARMS) PCR. Data were extracted from the HRPZ II database for analysis. Results Among the participants, 2327 individuals were found to have either common deletional (n=1037, 44.6%) or non-deletional (n=1290, 55.4%) α-thalassaemia. Hb Constant Spring was the most prevalent non-deletional α-thalassaemia, accounting for 53.03% of cases. Thirty-one participants (1.33%) exhibited αCD59α/αα, and one (0.04%) had αCD59α/-α3.7. Among the 32 subjects with Hb Adana, 87.5% were Malay, and 12.5% were Orang Asli. Additionally, seven cases of HbE/Hb Adana co-inheritance were identified. Hemoglobin levels in heterozygous Hb Adana individuals ranged from mild anemia to normal, between 95 g/L and 153 g/L. Mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH) were approximately 73 fL and 23 pg, respectively. Conclusion This study delineates the distribution of α-thalassaemia mutation patterns among high school students in Kelantan, Northeast Peninsular Malaysia. Our findings indicate that Hb Adana is rare in our region and co-inheritance with an α-gene deletion results in α+-thalassaemia and with HbE, α0-thalassaemia. All heterozygous Hb Adana individuals exhibited low MCVs and MCHs.
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