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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.
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.
Metabolic syndrome and antipsychotic monotherapy treatment among schizophrenia patients in Malaysia

Said MA, Hatim A, Habil MH, Zafidah W, Haslina MY, Badiah Y, et al.

Prev Med, 2013;57 Suppl:S50-3.
PMID: 23337566 DOI: 10.1016/j.ypmed.2013.01.005

OBJECTIVE: The objective of this study is to determine the prevalence of metabolic syndrome among schizophrenia patients receiving antipsychotic monotherapy in Malaysia.
METHOD: A cross-sectional study was conducted at multiple centres between June 2008 and September 2011. Two hundred and five patients who fulfilled the DSM IV-TR diagnostic criteria for schizophrenia and who had been on antipsychotic medication for at least one year, were screened for metabolic syndrome. Patients receiving a mood stabilizer were excluded from the study. Metabolic syndrome was defined by using the National Cholesterol Education Program (NCEP) Expert Panel on Detection, Evaluation, and Treatment of High Blood Cholesterol in Adults Treatment Panel III (ATP III) modified for Asian waist circumference.
RESULTS: In the first-generation antipsychotic (FGA) group, the highest prevalence of metabolic syndrome was among patients treated with trifluoperazine and flupenthixol decanoate (66.7% each). For the second-generation antipsychotic (SGA) group, the highest prevalence of metabolic syndrome was among patients treated with clozapine (66.7%). The component with the highest prevalence in metabolic syndrome was waist circumference in both FGA and SGA groups except for aripiprazole in SGA.
CONCLUSION: The prevalence of metabolic syndrome in schizophrenia patients receiving antipsychotic monotherapy in Malaysia was very high. Intervention measures are urgently needed to combat these problems.
KEYWORDS: Antipsychotics; Metabolic syndrome; Monotherapy; Prevalence; Schizophrenia
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.
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.
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.
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.
Para-Bombay blood group: Report of a rare blood group

Joibi KF, Noor NHM, Hassan MN, Iberahim S, Rahman WSWA, Yusoff SM, et al.

Asian J Transfus Sci, 2024;18(2):316-319.
PMID: 39822692 DOI: 10.4103/ajts.ajts_3_21

Para-Bombay blood group is a rare blood group typically characterized by the absence of H antigens on red blood cell and the presence of ABH substances on secretion. It can be easily missed and often mistaken as blood group O without extended testing. Detection is important as it significantly affect transfusion management. We report two cases of Para-Bombay A blood group in a teaching hospital in a North-Eastern state of Malaysia.
Fulltext Assessing the Diagnostic Value of Mean Monocyte Volume and Hematological Parameters in Predicting Dengue Fever: A Cross-Sectional Analysis

Ismail NH, Siddig A, Ab Aziz NAF, Ramli M, Zulkafli Z, Johan MF, et al.

Cureus, 2024 Dec;16(12):e75174.
PMID: 39759638 DOI: 10.7759/cureus.75174

BACKGROUND AND AIM: Distinguishing dengue fever (DF) from other viral infections solely based on common presentations poses a challenge. Therefore, there is a pressing need for additional diagnostic parameters that are reliable, swift, and cost-effective. This study aims to provide novel insights into the diagnostic value of hematological parameters, particularly mean monocyte volume (MMV), in predicting DF in Kelantan, Malaysia.
METHODOLOGY: This cross-sectional study enrolled 162 patients with suspected DF symptoms. The diagnosis was confirmed through dengue immunoglobulin M (IgM) capture enzyme-linked immunosorbent assay (ELISA) or Dengue Early ELISA for nonstructural protein 1 (NS1) antigen detection. Hematological parameters were measured using the Coulter DxH 800 hematology analyzer (Beckman Coulter, Brea, CA), and the statistical analysis was performed using SPSS version 22 (IBM Corp., Armonk, NY).
RESULTS: A total of 108 patients tested positive for DF, while 54 tested negative. We observed significant differences in WBC count, platelet count, and monocyte percentage between patients with DF and non-DF, while no significant correlation was noted for MMV. Subsequent statistical analysis, including receiver operating characteristic (ROC) curve analysis, revealed that monocyte percentage exhibited the largest area under the curve (0.715), indicating its potential as moderate discriminative power in diagnosing DF.
CONCLUSIONS: Our study findings indicate that monocyte percentage and MMV outcomes are insufficient for predicting DF, suggesting potential areas for further research.
Conformation sensitive gel electrophoresis for the detection of calreticulin mutations in BCR-ABL1-negative myeloproliferative neoplasms

Zakaria NA, Rosle NA, Siti Asmaa MJ, Aziee S, Haiyuni MY, Samat NA, et al.

Int J Lab Hematol, 2021 Dec;43(6):1451-1457.
PMID: 34125992 DOI: 10.1111/ijlh.13628

INTRODUCTION: Calreticulin (CALR) mutations in myeloproliferative neoplasms (MPN) have been reported to be key markers in the molecular diagnosis, particularly in patients lacking JAK2 V617F mutation. In most current reports, CALR mutations were analysed by either allele-specific PCR (AS-PCR), or the more expensive quantitative real-time PCR, pyrosequencing and next-generation sequencing. Hence, we report the use of an alternative method, the conformation sensitive gel electrophoresis (CSGE) for the detection of CALR mutations in BCR-ABL1-negative MPN patients.
METHODS: Forty BCR-ABL1-negative MPN patients' DNA: 19 polycythemia vera (PV), 7 essential thrombocytosis (ET) and 14 primary myelofibrosis (PMF), were screened for CALR mutations by CSGE. PCR primers were designed to amplify sequences spanning between exons 8 and 9 to target the mutation hotspots in CALR. Amplicons displaying abnormal CSGE profiles by electrophoresis were directly sequenced, and results were analysed by BioEdit Sequence Alignment Editor v7.2.6. CSGE results were compared with AS-PCR and confirmed by Sanger sequencing.
RESULTS: CSGE identified 4 types of mutations; 2 PMF patients with either CALR type 1 (c.1099_1150del52) or type 2 (c.1155_1156insTTGTC), 1 ET patient with nucleotide deletion (c.1121delA) and insertion (c.1190insA) and 1 PV patient with p.K368del (c.1102_1104delAAG) and insertion (c.1135insA) inframe mutations. Three patients have an altered KDEL motif at the C-terminal of CALR protein. In comparison, AS-PCR only able to detect two PMF patients with mutations, either type 1 and type 2.
CONCLUSION: CSGE is inexpensive, sensitive and reliable alternative method for the detection of CALR mutations in BCR-ABL1-negative MPN patients.
Fulltext A case of naturally occurring anti-Dia antibody in a young man

Iberahim S, Noor NHM, Hassan MN, Bahar R, Yusoff SM, Ramli M, et al.

Asian J Transfus Sci, 2022;16(2):276-279.
PMID: 36687544 DOI: 10.4103/ajts.ajts_136_21

The Diego (Di) blood group system comprises 22 antigens located on the band 3 protein, most of which are low-prevalence antigens. The majority of antibodies to Diego system antigens were of clinically insignificant; however anti-Dia, -Dib, -Wra, -ELO and-DISK may cause hemolytic disease of the fetus and newborn (HDFN) and transfusion reaction. We reported a case of naturally occurring of anti-Dia in a young man who presented to our hospital for wound debridement of fingers injury. His serological results were suggestive of anti-Dia antibody, and his molecular blood group showed he has Di (a-b+) antigen. Anti-Dia may be clinically significant. It can cause mild-to-severe HDFN, but there are only infrequent reports of it being clearly implicated in a hemolytic transfusion reaction. We suggest the need for reagent red blood cell panels to include Dia antigen-positive cells in antibody identification tests for our populations.
Fulltext The first Malay database toward the ethnic-specific target molecular variation

Halim-Fikri H, Etemad A, Abdul Latif AZ, Merican AF, Baig AA, Annuar AA, et al.

BMC Res Notes, 2015;8:176.
PMID: 25925844 DOI: 10.1186/s13104-015-1123-y

The Malaysian Node of the Human Variome Project (MyHVP) is one of the eighteen official Human Variome Project (HVP) country-specific nodes. Since its inception in 9(th) October 2010, MyHVP has attracted the significant number of Malaysian clinicians and researchers to participate and contribute their data to this project. MyHVP also act as the center of coordination for genotypic and phenotypic variation studies of the Malaysian population. A specialized database was developed to store and manage the data based on genetic variations which also associated with health and disease of Malaysian ethnic groups. This ethnic-specific database is called the Malaysian Node of the Human Variome Project database (MyHVPDb).

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