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Fulltext Apparent factor IX inhibitor

Jameela, S., Rozika, P., Rizalman, J., Phan, C.L., Visalachy, P., Chang, K.M.

Medicine & Health, 2011;6(2):126-130.
MyJurnal

The causes of an isolated prolonged activated partial thromboplastin time (APTT) with a normal prothrombin time (PT) are either a deficiency of clotting factors VIII, IX, XI or XII or the presence of an inhibitor. The inhibitor may be specific to an individual clotting factor or it may be a non-specific inhibitor like the lupus anticoagulant which has opposite therapeutic implications. We report a patient referred to our hospital for treatment that was previously diagnosed at another medical institution as an acquired factor IX inhibitor following an investigation for a prolonged APTT. On further testing this turned out to be a potent lupus anticoagulant which interfered with the phospholipid-dependent factor assays. The use of dilution studies, chromogenic assays and phospholipid neutralization can help differentiate these inhibitors. Great care must be taken in the interpretation of factor assays in the presence of lupus anticoagulant to avoid misdiagnosis and inappropriate treatment.

Matched MeSH terms: Factor VIII
Mutational Profiles of F8 and F9 in a Cohort of Haemophilia A and Haemophilia B Patients in the Multi-ethnic Malaysian Population

Zahari M, Sulaiman SA, Othman Z, Ayob Y, Karim FA, Jamal R

Mediterr J Hematol Infect Dis, 2018;10(1):e2018056.
PMID: 30210749 DOI: 10.4084/MJHID.2018.056

Background: Haemophilia A (HA) and Haemophilia B (HB) are X-linked blood disorders that are caused by various mutations in the factor VIII (F8) and factor IX (F9) genes respectively. Identification of mutations is essential as some of the mutations are associated with the development of inhibitors. This study is the first comprehensive study of the F8 mutational profile in Malaysia.
Materials and methods: We analysed 100 unrelated HA and 15 unrelated HB patients for genetic alterations in the F8 and F9 genes by using the long-range PCR, DNA sequencing, and the multiplex-ligation-dependent probe amplification assays. The prediction software was used to confirm the effects of these mutations on factor VIII and IX proteins.
Results: 44 (53%) of the severe HA patients were positive for F8 intron 22 inversion, and three (3.6%) were positive for intron one inversion. There were 22 novel mutations in F8, including missense (8), frameshift (9), splice site (3), large deletion (1) and nonsense (1) mutations. In HB patients, four novel mutations were identified including the splice site (1), small deletion (1), large deletion (1) and missense (1) mutation.
Discussion: The mutational spectrum of F8 in Malaysian patients is heterogeneous, with a slightly higher frequency of intron 22 inversion in these severe HA patients when compared to other Asian populations. Identification of these mutational profiles in F8 and F9 genes among Malaysian patients will provide a useful reference for the early detection and diagnosis of HA and HB in the Malaysian population.

Matched MeSH terms: Factor VIII
Fulltext A Self-Report Measure to Detect Neurocognitive Impairment among Incarcerated People Living with HIV in Malaysian Context: An Exploratory Factor Analysis

Shrestha R, Weikum D, Copenhaver M, Altice FL

Int J Ment Health Addict, 2017 Aug;15(4):812-825.
PMID: 29051714 DOI: 10.1007/s11469-017-9752-0

While neurocognitive impairment (NCI) among people living with HIV (PLWH) who use drugs and its impact on HIV treatment outcomes continue to be widely investigated, there remains a critical gap in NCI assessment among HIV-infected people who use drugs (PWUD) in Malaysian context. The present study, therefore, sought to improve the utility of the existing Neuropsychological Impairment Scale (NIS) as a screening tool for assessing NCI by establishing its psychometric properties in the Malaysian context. The NIS is a brief, self-report measure originally designed to assess NCI by having individuals rate a range of items that may influence cognitive functioning. HIV-infected male prisoners (N=301) administered the full 95-item NIS. An exploratory factor analysis (EFA) was conducted using principal axis factoring and orthogonal varimax rotation. Reliability was measured using Cronbach's alpha. The EFA resulted in an abbreviated, psychometrically sound, eight-factor structure (54-item) revised NIS - now referred to as the Brief Inventory of Neuro-cognitive Impairment-Malaysia (BINI-M). It is better designed to detect NCI in PLWH, ranging from generalized neurocognitive symptoms to more specific forms of impairment with excellent to average reliability. The BINI-M may serve as a useful tool for clinicians and researchers to assess NCI in PLWH and can inform enhanced treatment strategies in the Malaysian context.

Matched MeSH terms: Factor VIII
Shortened activated partial thromboplastin time, a hemostatic marker for hypercoagulable state during acute coronary event

Abdullah WZ, Moufak SK, Yusof Z, Mohamad MS, Kamarul IM

Transl Res, 2010 Jun;155(6):315-9.
PMID: 20478546 DOI: 10.1016/j.trsl.2010.02.001

Various factors may contribute to a hypercoagulable state and acute vascular thrombosis. A prospective study was conducted involving 165 coronary heart disease (CHD) patients from the Cardiology Unit, Hospital Universiti Sains Malaysia. The purpose of this study was to investigate the relationship among factor VIII (FVIII), prothrombin time (PT), activated partial thromboplastin time (APTT), and activated protein C resistance (APC-R) state among CHD patients and to look for potential clinical applications from these laboratory findings. There were 110 cases diagnosed as acute coronary syndrome (ACS), whereas another 55 were stable coronary artery disease (SCAD) patients. PT, APTT, FVIII, and APC-R assays were performed on all subjects. There was a significant difference between the FVIII level and the APTT results (P value < 0.0001). A negative relationship was found between the FVIII level and the APTT from linear regression analysis (R(2) = 10%, P value < 0.0001). For each 1% increase in the FVIII level, the APTT was reduced by 0.013 s (95% confidence interval (CI) between -0.019 and -0.007). Interestingly, none of the SCAD patients had abnormally short APTT. Approximately 68.4% of cases with a positive APC-R assay were found to have a high FVIII level. In conclusion, the APTT test is a potential hemostatic marker for hypercoagulable state including in arterial thrombosis.
Study site: Cardiology unit (outpatient and inpatient), Hospital Universisti Sains Malaysia (HUSM), Kelantan, Malaysia

Matched MeSH terms: Factor VIII/analysis
[Effect of Arvin on blood coagulation]

Vinazzer H

Wien Z Inn Med, 1971;52(8):378-92.
PMID: 5110844
Matched MeSH terms: Factor VIII/analysis
Fulltext Acquired Hemophilia of Unknown Etiology in an Elderly Man: Case Report

Stephen SE, Loong JLX, Hoong CK, Lim SM, Botross NP

Am J Case Rep, 2018 Jul 23;19:858-863.
PMID: 30033442 DOI: 10.12659/AJCR.909228

BACKGROUND Acquired hemophilia is a rare but potentially dangerous bleeding disorder caused by autoantibodies against coagulation factors. It affects 1 to 1.5 per 1 million people each year. While 50% of cases could be idiopathic, other causes include malignancies, diabetes, pregnancy, infection, and autoimmune disorders. CASE REPORT We report a case of a 90-year-old male who developed a spontaneous hematoma on the dorsum of his right hand, with no prior history of trauma or any other mucosal bleeding. His activated partial thromboplastin time (aPTT) was found to be prolonged (>180 seconds) with a very low level of factor VIII (0.1%). CONCLUSIONS As workups did not identify the source, including malignancy and autoimmune diseases, of his acquired hemophilia, it is believed to be idiopathic. He was started on intravenous recombinant factor VIIa (NovoSeven) to control the bleeding in combination with an immunosuppressive therapy of cyclophosphamide and prednisolone. In approximately 10% of patients with acquired hemophilia, underlying malignancy, such as squamous cell cancer, chronic lymphocytic leukemia, non-Hodgkin lymphoma, and multiple myeloma can present and commonly develop in elderly patients. Therefore, patients diagnosed with idiopathic acquired hemophilia should be given long-term follow up.

Matched MeSH terms: Factor VIII/analysis
Fulltext Failure to replicate the internal structure of Greek-specific thalassemia quality of life instrument in adult thalassemia patients in Sabah

Keowmani T, Lee LW

Patient Prefer Adherence, 2016;10:205-11.
PMID: 26955264 DOI: 10.2147/PPA.S96880

PURPOSE: To study the validity and reliability of the Malay version of the Specific Thalassemia Quality of Life Instrument (STQOLI) in Sabah's adult thalassemia patients.
PATIENTS AND METHODS: This cross-sectional study was done at Thalassemia Treatment Centre, Queen Elizabeth Hospital in Sabah, Malaysia. Eighty-two adult thalassemia patients who fulfilled the inclusion and exclusion criteria were conveniently selected for participation in the study. The English version of STQOLI was translated into Malay by using forward and back translations. The content of the questionnaire was validated by the chief hematologist of the hospital. The construct validity of the 40-item questionnaire was assessed by principal component analysis with varimax rotation and the scale reliability was assessed by Cronbach's alpha.
RESULTS: The study failed to replicate the internal structure of the Greek STQOLI. Instead, 12 factors have been identified from the exploratory factor analysis, which accounted for 72.2% of the variance. However, only eight factors were interpretable. The factors were iron chelation pump impact, transfusion impact, time spent on treatment and its impact on work and social life, sex life, side effects of treatment, cardiovascular problems, psychology, and iron chelation pill impact. The overall scale reliability was 0.913.
CONCLUSION: This study was unable to replicate the internal structure of the Greek STQOLI in Sabah's adult thalassemia patients. Instead, a new structure has emerged that can be used as a guide to develop a questionnaire specific for adult thalassemia patients in Sabah. Future research should focus on the eight factors identified from this study.
KEYWORDS: Malay; STQOLI; reliability; transfusion; validity

Matched MeSH terms: Factor VIII
Comparative analysis of chromogenic vs clot.based one stage APTT assay for determination of factor VIII level

Baig MA, Swamy KB

Indian J Pathol Microbiol, 2021 1 13;64(1):123-127.
PMID: 33433421 DOI: 10.4103/IJPM.IJPM_900_19

Background: In the laboratory, factor VIII can be measured by three different methodologies, such as one-stage clotting assay, two-stage clotting assay, and chromogenic assay. These assays differ in ease of use, variety of reagents available, sensitivity to mild hemophilia A, and interference from lupus anticoagulants (LACs). Certain factor VIII gene mutations can cause discrepancy in results between one-stage activated partial thromboplastin time (APTT) and chromogenic assays.
Materials and Methods: The coagulometer for factor VIII assay is Sysmex CS-5100. All data were expressed as mean ± standard deviation (SD).
Results: A total of 135 cases were studied. Of these, 100 cases were of mild hemophilia A diagnosed by molecular genetics and, 15 cases were positive for LAC, which were confirmed by dilute Russell Viper venom test. Clot-based one-stage APTT assay showed 65% sensitivity and 80% specificity in diagnosing mild hemophilia A cases and out of 15 LAC cases, it showed false positivity in five cases. Chromogenic assay showed 85% sensitivity and 90% specificity in diagnosing mild hemophilia cases and was 100% specific in excluding LAC cases.
Conclusions: One-stage APTT assay is the most commonly used test for determining factor VIII levels but chromogenic assay are considered as the gold standard and recommended as the reference method by European Pharmacopoeia and ISTH subcommittee. Mild hemophilia A patients with missense mutations show discrepancy between the one-stage clot-based APTT assay and chromogenic assays for determination of factor VIII level and this can lead to misdiagnosis or misclassification of mild hemophilia A. Therefore, it is recommended that both the assays should be used in the evaluation of mild hemophilia cases.

Matched MeSH terms: Factor VIII
Fulltext Development and validation of the maternal blues scale through bonding attachments in predicting postpartum blues

Manurung S, Setyowati S

Malays Fam Physician, 2021 Mar 25;16(1):64-74.
PMID: 33948144 DOI: 10.51866/oa1037

Background: Postpartum blues in Indonesia has a high prevalence at 37% to 67%. Postpartum blues syndrome has been described as varying changes in the affective domain, such as feelings, behavior, or thoughts, that can be influenced by the roles and tasks of women, along with their social, cultural, and economic support. Instruments that measure maternal blues through bonding attachment behavior have never before been developed in Indonesia.
Objective: This study aimed to develop a maternal blues scale through bonding attachments to predict postpartum blues.
Method: The research design consisted of three stages: 1) phenomenology design and focus group discussion; 2) development and construction of the maternal blues scale, and 3) a cross-sectional study to measure validation of the scales. Respondents were postpartum mothers in the first week after birth. The sample comprised 501 participants. Sampling was done by consecutive sampling at the Public Health Center (PUSKESMAS) in the South Jakarta area. Data analysis used exploratory factor analysis (EFA) and confirmatory factor analysis (CFA), correlation, and a diagnostic testing .
Results: Item analysis produced 32 items consisting of 24 items regarding the mother's role and duties as internal factors and eight factors involving social, cultural, and economic support as external factors. Both factors were valid and reliable in predicting postpartum blues with indicators (t loading factors ≥ 1.96, standardized loading factor (SLF) ≥.50, internal factors: construct reliability (CR) ≥ .70 and extraction variants (VE) ≥ .50 and external factors: CR ≥ .74 to .83 VE ≥ .50 to .63). The relationship with Kennerley's maternity blues as a gold standard was significant. Internal factors had a score of 53, with a sensitivity of 60.2%. The external factors score was 19, with a sensitivity of 77.3%.
Conclusion: The new scale for postpartum blues prediction developed displayed internal consistency and validity of each indicator (internal and external factors) that was good (CR ≥ .70; VE ≥ .50). This scale provides a feasible tool to predict postpartum blues.

Matched MeSH terms: Factor VIII
Fulltext Von Willebrand factor profiles of the different ABO blood groups among Malay population

Rohaida Abd Rahman, Faridah Afandi, Tun Maizura Mohd Fathullah, Rafeezul Mohamed

Journal of Biomedical and Clinical Sciences, 2017;2(202):40-41.
MyJurnal

The National Blood Center, Kuala Lumpur interprets laboratory results for the von Willebrand factor (vWF) profile based on guidelines provided by the U.S. National Heart, Lung, and Blood Institute, which were established based on the Caucasian population [1-2]. The vWF profiles among the Malay population has not yet been established.

The goals of this study were to determine the vWF profiles of the different ABO blood types among Malays and to evaluate their association with demographic characteristics and smoking habits.

One hundred and forty Malay donors participated in this study. Factor VIII (FVIII), vWF antigen, and ristocetin cofactor (RiCof) levels and collagen binding activity (CBA) were measured by coagulometric clot detection, latex agglutination, and enzyme-linked immunosorbent assay.

Matched MeSH terms: Factor VIII