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Sickle cell anemia associated with alpha-thalassemia in Malaysian Indians

Lie-Injo LE, Hassan K, Joishy SK, Lim ML

Am J Hematol, 1986 Jul;22(3):265-74.
PMID: 2424302

The Indian rubber estate workers in Negri Sembilan, Malaysia, who originated from Orissa in India were found to have a high frequency of Hb S (Joishy SK, Hassan K: Clin Res 28:280, 1980). Unlike the usually severe clinical picture of sickle cell anemia seen in African and American blacks, the clinical picture of the disease in this population was mild and many have reached old age. We studied the leukocyte DNA of 12 patients with sickle cell anemia, ranging in age from 4 to 61 years and 30 sickle cell trait carriers, ranging in age from 7 to 63 years, for the presence of alpha-globin gene deletions by gene mapping according to Southern (Southern EM: J Mol Biol 98:503, 1975), using alpha- and zeta-globin gene probes obtained by nick translation of the alpha- and zeta-globin genes cloned into plasmid. All 12 sickle cell anemia patients were found to have alpha-thalassemia2 (alpha-thal2), either in the homozygous or heterozygous condition. Of the Hb S trait carriers, six did not have alpha-thal2 or alpha-thal1 and 24 had alpha-thal2 (15 heterozygous, 9 homozygous). Seven of these Hb S trait carriers with alpha-thal2 had an additional gene abnormality. Five of them had a fast-moving Eco RI fragment 5.6 kb long that hybridized with zeta-specific probe but not with alpha-specific probe. An unusual DNA pattern of a different type was further found in the other two. Bgl II restriction analysis showed that the alpha-thal2 was mostly of the rightward deletion alpha-thal1 genotype. None of the sickle cell anemia patients and Hb S trait carriers had deletion type alpha-thal1. The sickle cell anemia patients had very high levels of Hb F and low levels of Hb A2. The Hb S trait carriers with alpha-thal2 had relatively low levels of Hb S.

Matched MeSH terms: Hemoglobins/metabolism
Severe iron deficiency anaemia in prolonged exclusively breast and cow's milk-fed infants

Haifa Abdul Latiff, Mohd Nasir Hassan, Mohd Sham Kasim

Malaysian Journal of Child Health, 1994;6(1):49-51.
MyJurnal

We report here a case of severe iron deficiency anaemia resulting from prolonged exclusive milk diet without any iron supplementation. The patient was a 1112 year old boy, the only child of a factory worker, who was breasffed until 4 months of age followed by exclusive feeding by non-fortifiedformula. He presented with severe iron deficiency anaemia with haemoglobin of4 .0g% and required blood trans-fusion. Iron and.vitamin supplements were given and cereals were slowly introduced. He responded very well to the treatment with satis-factory weight gain and improvement of anaemia. (Copied from article).

Matched MeSH terms: Hemoglobins
Fulltext Serum soluble transferrin receptor concentration as a biomarker of erythropoietic activity: surrogate marker of adequate transfusion in adult beta-thalassaemia intermedia patients

Thambiah, S., George, E., Nor Aini, U., Sathar, J., Zarida, H., Mokhtar, A.B.

Malaysian Journal of Medicine and Health Sciences, 2013;9(2):3-12.
MyJurnal

Management of Beta (β)-thalassaemia intermedia in contrast to β-thalassaemia major patients has no clear guidelines as to indicators of adequate transfusion. Regular blood transfusion suppresses bone marrow erythropoietic activity. Serum soluble transferrin receptor (sTfR) concentration is a marker for erythropoietic activity, with increased sTfR being associated with functional iron deficiency and increased erythropoietic activity. This study aimed to determine the use of sTfR as an indicator of adequate transfusion in adult β-thalassaemia intermedia patients. A cross-sectional study was conducted at Hospital Ampang, Malaysia, for six months. Patient group included six β-thalassaemia intermedia and 34 HbE-β-thalassaemia transfused patients. None of the patients were on regular monthly blood transfusions as in β-thalassaemia major. The control group comprised of 16 healthy subjects with normal haematological parameters. Haemoglobin (Hb) analysis, sTfR and ferritin assays were performed. Hb and HbA percentages (%) were found to be significantly lower in patients compared to the controls, while HbE%, HbF%, sTfR and ferritin were significantly higher in patients. An inverse relationship was found in the controls between HbF% with Hb (r = -0.515, p < 0.05) and HbA% (r = -0.534, p < 0.05). In patients, sTfR showed an inverse relationship with HbA% (r = -0.618, p = 0.000) and a positive correlation with HbE% (r = 0.418, p = 0.007) and HbF% (r = 0.469, p = 0.002). Multivariate analysis showed that HbA% (r = 2.875, p = 0.048), HbE% (r = 2.872, p = 0.020) and HbF% (r = 2.436, p = 0.013) best predicted sTfR independently in patients. Thus, sTfR is a useful marker for erythropoiesis. The elevated sTfR in these patients indicate that the transfusion regimen used was inadequate to suppress ineffective erythropoiesis. Hb levels may not be the best target for monitoring transfusion treatment in β-thalassaemia intermedia patients, but the use of sTfR is helpful in individualising transfusion regimens.

Matched MeSH terms: Hemoglobins
Serum ferritin in haemoglobin H

George E, Faridah K

Family Practitioner, 1986;9:38-40.

Matched MeSH terms: Hemoglobins
Serum ferritin concentrations in male Malaysian blood donors

Ton SH, Lopez CG

Southeast Asian J. Trop. Med. Public Health, 1980 Mar;11(1):87-90.
PMID: 7403958

Serum ferritin and haemoglobin estimates were carried out on 78 first time blood donors with a view to determining iron store status. Of these 30 were Malays, 20 were Chinese and 28 were Indians. The ferritin level in Malay donors ranged from 16-160 mg/ml (mean 83 +/- 49.4 mg/ml in chinese donors is ranged from 36-500 mg/ml (mean 242.8 +/- 132 mg/ml), and in the Indian donors it ranged from 5 - 270 mg/ml (mean 94.6 +/- 67.9 mg/ml). The haemoglobin concentration for the whole group was 14.9 +/- 1.49 g/dl. There was no correlation of haemoglobin concentration with serum ferritin levels.

Matched MeSH terms: Hemoglobins/analysis
Fulltext Secondary polycythaemia in a Malay girl with homozygous Hb Tak

Amran HS, Aziz MA, George E, Mahmud N, Lee TY, Md Noor S

Malays J Pathol, 2017 Dec;39(3):321-326.
PMID: 29279598 MyJurnal

Hb Tak is one of more than 200 high affinity haemoglobin variants reported worldwide. It results from the insertion of two nucleotides (AC) at the termination codon, between codon 146 and codon 147 of the beta-globin gene [Beta 147 (+AC)]. Polycythaemia is the main clinical feature although affected carriers are usually asymptomatic and do not require intervention. Several case studies in this region have reported the co-inheritance of Hb Tak with Hb E, delta beta and beta thalassaemia with one case of homozygous Hb Tak in a Thai boy. In this case report, a cluster of haemoglobin Tak was found in a family of Malay ethnic origin. Cascade family screening was conducted while investigating a 4-year old girl who presented with symptomatic polycythaemia. She had 2 previous Hb analysis done, at 7-month and 2-year-old with the diagnosis of possible Hb Q Thailand and Homozygous Hb D, respectively. Both diagnosis did not fit her clinical presentations. She was plethoric, had reduced exercise tolerance as well as cardiomyopathy. Her parents were consanguineously married and later diagnosed as asymptomatic carriers of Hb Tak. Consequently, re-analysis of the girl's blood sample revealed a homozygous state of Hb Tak. In conclusion, high oxygen affinity haemoglobin like Hb Tak should be considered in the investigation of polycythaemic patients with abnormal Hb analyses. In this case, DNA analysis was crucial in determining the correct diagnosis.

Matched MeSH terms: Hemoglobins, Abnormal/genetics*
Fulltext Screening of concurrent alpha-thalassaemia 1 in beta-thalassaemia carriers

Chong YM, Tan JA, Zubaidah Z, Rahimah A, Kuldip K, George E

Med J Malaysia, 2006 Jun;61(2):217-20.
PMID: 16898315

Thalassaemia is an inherited blood disorder and is a significant public health problem in Malaysia, with many not knowing they carry the gene for thalassaemia. The two major forms are alpha and beta thalassaemia. An individual can co-inherit both the alpha and beta thalassaemia genes. This study determined the frequency of concurrent carriers of alpha thalassaemia in 231 beta thalassaemia carriers. Gap-PCR was done on extracted DNA of the beta thalassaemia samples to check for alpha thalassaemia 1 molecular defect. Eight (3.5%) samples were found to have concurrently inherited the alpha thalassaemia 1 (- -SEA) deletion. The significant carrier rate for alpha thalassaemia 1 indicates the need for the implementation of DNA analysis to complement thalassaemia screening in high risk populations.

Matched MeSH terms: Hemoglobins/metabolism
Fulltext Screening for thalassaemia among group of students of a higher institution – our experience

Norlelawati, A.T., Siti Hadijah, M., Siti Nor Haiza, H., Rusmawati, I., Abdul Wahab, J., Naznin, M., et al.

International Medical Journal Malaysia, 2011;10(1):3-6.
MyJurnal

Introduction: Thalassaemia is an inherited blood disorder and is a significant public health alarm in Malaysia with many not knowing they are carriers of this haemoglobin disorders. Materials and methods: This study conducted a one off collection of blood samples from 72 Malays students of International Islamic University Malaysia (IIUM) in Kuantan. Blood samples were subjected to conventional haemoglobin analyses that include full blood count and picture, HPLC, Haemoglobin electrophoresis and H-inclusion test. All samples were also genotyped for alpha thalassaemia–1 of Southeast Asia (a-Thal1SEA). Result: There were 17(23.6%) students who were diagnosed as thalassaemia carriers. Out of this, four (5.5 %) and six (8.3 %) students were presumptive β-thalassaemia trait and Haemoglobin-E trait as determined by the HPLC assay respectively. Nine (12.5%) students were genotyped a-Thal1SEA among whom two were also β-thalassaemia carriers. All thalassaemia cases had MCH of < 27pg. Nonetheless, two out of six Haemoglobin-E trait and three out of nine a-Thal1SEA carrier had MCV value of >80fL. Two out of four (50%) presumptive β -thalassaemia trait and one out of six (17%) students of presumptive Haemoglobin-E trait had family history of thalassaemia respectively. Conclusion: The high occurrence of the three common types of thalassaemia carrier (β, Hb-E and a-Thal1SEA thalassaemia) in our small group of subjects could be due to better participation of students who had family history of thalassaemia. The study reaffirmed the importance of molecular study for detection of alpha-thalassaemia and the use of MCH value of

Matched MeSH terms: Hemoglobins
Fulltext School-administered weekly iron-folate supplements improve hemoglobin and ferritin concentrations in Malaysian adolescent girls

Tee ES, Kandiah M, Awin N, Chong SM, Satgunasingam N, Kamarudin L, et al.

Am J Clin Nutr, 1999 Jun;69(6):1249-56.
PMID: 10357747 DOI: 10.1093/ajcn/69.6.1249

BACKGROUND: Iron deficiency and its consequent anemia constitute the commonest micronutrient deficiency in the world.
OBJECTIVE: We investigated whether long-term, weekly iron-folate supplements administered at school would improve hemoglobin and ferritin concentrations in adolescent girls, including those with mild-to-moderate anemia and hemoglobin concentrations indicating borderline anemia.
DESIGN: Subjects were 266 girls with hemoglobin concentrations of 80-119.9 g/L (group A) and 358 girls with hemoglobin concentrations of 120-130 g/L (group B) who were otherwise healthy. Two hundred sixty-six girls in group A and 268 girls in group B were randomly assigned to receive either 60 or 120 mg Fe plus 3.5 mg folic acid weekly for 22 wk. Ninety of the girls in group B were randomly assigned to receive only 5 mg folic acid weekly. Capillary hemoglobin and plasma ferritin were measured at baseline and after 12 and 22 wk of supplementation.
RESULTS: By the end of the study, 2% of the girls had dropped out and > 96% had taken > or = 20 of the 22 tablets; side effects were minimal. Mean plasma ferritin increased significantly in all iron-supplemented groups, independently of initial hemoglobin values and iron doses. Ferritin concentrations decreased in the girls supplemented with folic acid only. As expected, hemoglobin responses to iron were higher in group A than in group B and increases were positively correlated with initial plasma ferritin. Hemoglobin failed to respond to folate supplementation if initial plasma ferritin concentrations were low. Mean hemoglobin increased significantly and consistently in relation to the length of treatment.
CONCLUSION: Long-term, weekly iron-folate supplementation was found to be a practical, safe, effective, and inexpensive method for improving iron nutrition in adolescent schoolgirls.

Comment in: Picciano MF. Iron and folate supplementation: an effective intervention in
adolescent females. Am J Clin Nutr. 1999 Jun;69(6):1069-70. PubMed PMID:
10357724. https://academic.oup.com/ajcn/article/69/6/1069/4714856

Study site: 3 secondary schools, Samarahan district, Sarawak, Malaysia

Matched MeSH terms: Hemoglobins/analysis*
Safety and Efficacy of Pegcetacoplan in Adult Patients with Paroxysmal Nocturnal Hemoglobinuria over 48 Weeks: 307 Open-Label Extension Study

Patriquin CJ, Bogdanovic A, Griffin M, Kelly RJ, Maciejewski JP, Mulherin B, et al.

Adv Ther, 2024 May;41(5):2050-2069.
PMID: 38573482 DOI: 10.1007/s12325-024-02827-8

INTRODUCTION: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, life-threatening disease characterized by complement-mediated hemolysis and thrombosis. Pegcetacoplan, the first targeted complement component 3 (C3) PNH therapy, was safe and efficacious in treatment-naive and pre-treated patients with PNH in five clinical trials.
METHODS: The 307 open-label extension (OLE) study (NCT03531255) is a non-randomized, multicenter extension study of long-term safety and efficacy of pegcetacoplan in adult patients with PNH who completed a pegcetacoplan parent study. All patients received pegcetacoplan. Outcomes at the 48-week data cutoff (week 48 of 307-OLE or August 27, 2021, whichever was earlier) are reported. Hemoglobin concentrations, Functional Assessment of Chronic Illness Therapy (FACIT)-Fatigue scores, and transfusion avoidance were measured. Hemoglobin > 12 g/dL and sex-specific hemoglobin normalization (i.e., male, ≥ 13.6 g/dL; female, ≥ 12 g/dL) were assessed as percentage of patients with data available and no transfusions 60 days before data cutoff. Treatment-emergent adverse events, including hemolysis, were reported.
RESULTS: Data from 137 patients with at least one pegcetacoplan dose at data cutoff were analyzed. Mean (standard deviation [SD]) hemoglobin increased from 8.9 (1.22) g/dL at parent study baseline to 11.6 (2.17) g/dL at 307-OLE entry and 11.6 (1.94) g/dL at data cutoff. At parent study baseline, mean (SD) FACIT-Fatigue score of 34.1 (11.08) was below the general population norm of 43.6; scores improved to 42.8 (8.79) at 307-OLE entry and 42.4 (9.84) at data cutoff. In evaluable patients, hemoglobin > 12 g/dL occurred in 40.2% (43 of 107) and sex-specific hemoglobin normalization occurred in 31.8% (34 of 107) at data cutoff. Transfusion was not required for 114 of 137 patients (83.2%). Hemolysis was reported in 23 patients (16.8%). No thrombotic events or meningococcal infections occurred.
CONCLUSION: Pegcetacoplan sustained long-term improvements in hemoglobin concentrations, fatigue reduction, and transfusion burden. Long-term safety findings corroborate the favorable profile established for pegcetacoplan.
TRIAL REGISTRATION: ClinicalTrials.gov identifier, NCT03531255.

Matched MeSH terms: Hemoglobins/analysis
Safety against nephrotoxicity in paclitaxel treatment: Oral nanocarrier as an effective tool in preclinical evaluation with marked in vivo antitumor activity

Choudhury H, Gorain B, Tekade RK, Pandey M, Karmakar S, Pal TK

Regul Toxicol Pharmacol, 2017 Dec;91:179-189.
PMID: 29080846 DOI: 10.1016/j.yrtph.2017.10.023

Oral paclitaxel (PTXL) formulations freed from cremophor® EL (CrEL) is always in utmost demand by the cancerous patients due to toxicities associated with the currently marketed formulation. In our previous investigation [Int. J. Pharm. 2014; 460:131], we have developed an oral oil based nanocarrier for the lipophilic drug, PTXL to target bioavailability issue and patient compliance. Here, we report in vivo antitumor activity and 28-day sub-chronic toxicity of the developed PTXL nanoemulsion. It was observed that the apoptotic potential of oral PTXL nanoemulsion significantly inhibited the growth of solid tumor (59.2 ± 7.17%; p

Matched MeSH terms: Hemoglobins/metabolism
Fulltext Risk factors associated with nosocomial infections among end stage renal disease patients undergoing hemodialysis: A systematic review

Abbasi SH, Aftab RA, Chua SS

PLoS One, 2020;15(6):e0234376.
PMID: 32569265 DOI: 10.1371/journal.pone.0234376

BACKGROUND: Profound healthcare challenges confront societies with an increase in prevalence of end-stage renal disease (ESRD), which is one of the leading causes of morbidity and mortality worldwide. Due to several facility and patient related factors, ESRD is significantly associated with increased morbidity and mortality attributed to infections.
AIMS AND OBJECTIVE: The aim of this study was to assess systematically the characteristics of patients and risk factors associated with nosocomial infections among ESRD patients undergoing hemodialysis.
METHODS: A systematic literature search was performed to identify eligible studies published during the period from inception to December 2018 pertaining to risk factors associated with nosocomial infections among hemodialysis patients. The relevant studies were generated through a computerized search on five databases (PubMed, EBSCOhost, Google Scholar, ScienceDirect and Scopus) using the Mesh Words: nosocomial infections, hospital acquired infections, healthcare associated infections, end stage renal disease, end stage renal failure, hemodialysis, and risk factors. The complete protocol has been registered under PROSPERO (CRD42019124099).
RESULTS: Initially, 1411 articles were retrieved. Out of these, 24 were duplicates and hence were removed. Out of 1387 remaining articles, 1337 were removed based on irrelevant titles and/or abstracts. Subsequently, the full texts of 50 articles were reviewed and 41 studies were excluded at this stage due to lack of relevant information. Finally, nine articles were selected for this review. Longer hospital stay, longer duration on hemodialysis, multiple catheter sites, longer catheterization, age group, lower white blood cell count, history of blood transfusion, and diabetes were identified as the major risk factors for nosocomial infections among hemodialysis patients.
CONCLUSION: The results of this review indicate an information gap and potential benefits of additional preventive measures to further reduce the risk of infections in hemodialysis population. Moreover, several patient-related and facility-related risk factors were consistently observed in the studies included in this review, which require optimal control measures.

Matched MeSH terms: Hemoglobins/metabolism
Risk association, linkage disequilibrium and haplotype analyses of FASN rs4246445, rs2229425, rs2228305 and rs2229422 polymorphisms in overweight and obesity

Chong ETJ, Kuok SSE, Lee PC

Bioimpacts, 2018;8(3):159-165.
PMID: 30211075 DOI: 10.15171/bi.2018.18

Introduction:
Obesity is commonly linked up with several life-threatening diseases. This study aims to investigate the association of fatty acid synthase (FASN) rs4246445, rs2229425, rs2228305, and rs2229422 single nucleotide polymorphisms (SNPs) with the risk of overweight and obesity in the Malaysian population.
Methods:
Blood samples were collected from 1030 individuals who were grouped into normal, overweight, and obese categories. Blood biochemistry test and lipid profiling were performed and genomic DNA was extracted. Genotyping was performed using hydrolysis probes and odd ratio with 95% CI was calculated for risk association analysis. Linkage disequilibrium and haplotypes analyses were carried out using SHEsis software.
Results:
We found that the hemoglobin and white blood cell counts were significantly high in the obese subjects. There is a lack of evidence to link the FASN SNPs with the risk of overweight and obesity in the population. All 4 SNPs were seemed to be in linkage equilibrium. Five common haplotypes were identified in this study but none of them was significantly associated with overweight and obesity in the population.
Conclusion:
Our findings suggest a lack of evidence to associate the FASN rs4246445, rs2229425, rs2228305, and rs2229422 SNPs with the risk of overweight and obesity in the Malaysian population. All 4 SNPs were independent of each other and not all identified haplotypes were significantly associated with overweight and obesity in this study.

Matched MeSH terms: Hemoglobins
Retinal findings in adult leukaemia: correlation with leukocytosis

Jackson N, Reddy SC, Hishamuddin M, Low HC

Clin Lab Haematol, 1996 Jun;18(2):105-9.
PMID: 8866143

The associations between retinal findings and haematological parameters in acute leukaemia are controversial. Sixty-three newly-diagnosed acute leukaemia patients, aged 12-77 years, were studied prospectively for the presence of intra-retinal haemorrhages (IRH), white-centred haemorrhages (WCH), cotton wool spots (CWS) and macular haemorrhages (MH), Thirty-three patients (52.4%) showed at least one retinal abnormality. The prevalence of individual findings was: IRH (30 cases), WCH (20 cases), CWS (5 cases), MH (11 cases). In contrast to previous studies, there was no association between any of these retinal findings and the haemoglobin level or the platelet count. There was a higher median WBC in patients with IRH (68 x 10(9)/l) than in those without IRH (15.4 x 10(9)/l), P = 0.037. When the acute myeloblastic leukaemia cases were considered separately, an association was also found between higher WBC and the presence of WCH and CWS. There was no association between retinal findings and FAB type in the AML cases. We conclude that a high WBC may be at least as important as anaemia and thrombocytopenia in the pathogenesis of the retinopathy of acute leukaemia.

Matched MeSH terms: Hemoglobins/analysis
Fulltext Reticulocyte haemoglobin as a biomarker for the detection of iron deficiency anaemia in haemodialysis patients on recombinant human erythropoietin

Jamian, E., Sanip, Z., Ramli, M., Mohd Daud, K., Mohamad, S., Hassan, R.

Journal of Biomedical and Clinical Sciences, 2018;3(2):29-34.
MyJurnal

Iron deficiency anaemia (IDA) frequently occurs in haemodialysis
(HD) patients undergoing recombinant human erythropoietin (rHuEPO)
therapy and is commonly associated with rHuEPO hypo-responsiveness.
However, the conventional iron indices are inadequate to exhibit the status or
utilisation of iron during erythropoiesis. The aim of this study was to elucidate
the accuracy and usefulness of the reticulocyte haemoglobin (RET-He) test
for diagnosing IDA in HD patients undergoing rHuEPO therapy. Methods: In
this cross-sectional study, fifty-five blood samples of HD patients on rHuEPO
therapy were collected and analysed for haematological and biochemical
parameters. A receiver operating characteristics curve was also plotted for
sensitivity and specificity analysis. IDA detection rates by RET-He, soluble
transferrin receptor (sTfR) and serum ferritin were 63.64%, 3.64% and 0%,
respectively. RET-He level was significantly correlated with sTfR level, mean
cell volume, mean cell haemoglobin level and the transferrin receptor-ferritin
index. The sensitivity and specificity of RET-He in detecting IDA were 78.3%
and 92.0%, respectively, with an area under the curve of 0.864. IDA was more
frequently detected by RET-He than by ferritin or sTfR in HD patients
undergoing rHuEPO therapy. The RET-He level also showed higher sensitivity
and specificity for the iron status in these patients. Therefore, RET-He is a
useful biomarker for the detection of IDA in HD patients undergoing rHuEPO
therapy.

Matched MeSH terms: Hemoglobins
Fulltext Relative proteome quantification of alpha, beta, gamma and delta globin chains in early eluting peaks of Bio-Rad variant II® CE-HPLC of hemoglobin from healthy and beta-thalassemia subjects in Malaysia

Abdullah UYH, Ibrahim HM, Jassim HM, Salleh MZ, Kek TL, Fakhruzzaman Bin Noorizhab MN, et al.

Biochem Biophys Rep, 2019 Jul;18:100635.
PMID: 31061897 DOI: 10.1016/j.bbrep.2019.100635

This is the first report of QQQ-mass spectrometric identification and quantification of the Hb subunits, alpha, beta, delta and gamma globin peptides, derived from enzymatic-digestion of proteins in the early unknown peaks of the Bio-Rad cation-exchange chromatography of haemoglobin. The objectives were to assess the relationship of the quantity of the free alpha, beta, delta and gamma globin chains with the phenotypic diversity of beta-thalassaemias (β-thal). The results demonstrate that the pools of free globin chains in red blood cells were correlating with the severity of the disease in patients with different phenotypes of β-thal. The mechanism and the regulation of synthesis of free globin chains pool in a normal individual and in patients with different β-thal phenotypes could arise from several mechanisms which will require further investigation. The role of the free globin pool in patients with β-thal for development of novel therapeutic approaches based on these potential targets requires further investigation. Pertinent biomarkers improves the diagnosis of the β-thal, especially in low-income countries where they are most common and allows more effective therapeutic intervention leading to more successful therapeutic outcome.

Matched MeSH terms: Hemoglobins
Fulltext Relationship of anaemia with cognitive and functional status of elderly in Rembau, Negeri Sembilan. [Hubungan anemia dengan status kognitif dan kefungsian warga tua di Rembau, Negeri Sembilan]

Aris N, Abdul Rahman S, Shahidan N

Sains Malaysiana, 2009;38(6):953–958.

The prevalence of anaemia and nutritional status was evaluated among 88 Malay elderly (20 men and 68 women) aged 60 to 85 years (mean age 69.8 ± 6.0 years) from four villages in Rembau district, Negeri Sembilan. In addition, the relationship between hemoglobin with nutrient intake, cognitive and functional status of the elderly were also investigated. Subjects were interviewed to obtain information on demographic and nutrient intake. Cognitive status was assessed using Elderly Cognitive Assessment Questionnaire (ECAQ) while functional status was measured using Instrumental Activity Daily Living (IADL) and hand grip measurement. Hemoglobin level was determined using HemoCue method. The findings indicated that the prevalence of anaemia was 22.7%. Prevalence of anaemia for male subject was 30.0% with mean of hemoglobin as 11.7 ± 1.0 g/dL while 20.6% of female subject was anaemic with mean of hemoglobin was 11.2 ± 0.5 g/dL. As much as 21.6% of the subjects have cognitive impairment with the prevalence is high in old-old age group (57.9%) compared to the young-old age group (11.6%). Results from functional assessment showed that mean for IADL score as 11 ± 3. The IADL score was lower in old-old age group (9 ± 4) compared to the young-old age group (12 ± 2). For hand grip measurement, overall mean was 16.8 ± 8.7 kg (14.2 ± 8.4 kg for old-old age group and 17.6 ± 8.7 kg for young-old age group). Nutrient analysis showed that the mean calorie intake for men (1310 ± 448 kcal/day) and women (1180 ± 300 kcal) were lower than the RNI. However, only intakes of iron, niacin and vitamin A achieved the Malaysian Recommended Nutrient Intake (RNI). Correlation between hemoglobin and nutrients was only showed with calorie intake (r=0.486, p=0.048) and not with other nutrients. Besides that, there was no correlation between hemoglobin with ECAQ and IADL scores but hemoglobin was correlated with hand grip strength (r=0.265, p=0.013). As a conclusion, 22.7% case of anaemia was reported in this study. However, correlations were only formed between hemoglobin with calorie intake and hemoglobin with hand grip. Anaemia in elderly increases the inability of the elderly to live independently.
Keywords: Anemia; cognitive status; elderly; functional status

Matched MeSH terms: Hemoglobins
Fulltext Reduction of tear-drop poikilocytes in a case of myelofibrosis following splenectomy

Chooi, Fun Leong, Soon, Keng Cheong

Medicine & Health, 2006;1(1):81-84.
MyJurnal

Myelofibrosis is a chronic myeloproliferative disorder characterised by splenomegaly, leukoerythroblastic anaemia, tear-drop poikilocytosis and marrow fibrosis. Splenectomy is indicated for patients requiring frequent transfusions or massive splenomegaly causing distressing symptoms and severe thrombocytopenia secondary to hypersplenism.A 56 year-old lady diagnosed to have primary myelofibrosis in year 2000 was referred to HUKM for further management of her increasing requirement of blood transfusion and massive splenomegaly. She has had two episodes of acute cholecytitis. Investigations done in our hospital showed that her hemoglobin level was 6.4g/dl, white cell count of 23.4x109/l, platelet count 163x109/l and peripheral blood showing leukoerythroblastic picture and tear-drop poikilocytosis. Leukocyte alkaline phosphatase score (NAP) was 184/100 neutrophils. Ultrasound of the abdomen showed massive splenomegaly with multiple gall stones. In view of the frequent transfusion requirements and symptomatic massive splenomegaly, splenectomy and cholecystectomy was performed. A review of her peripheral blood picture, post-splenectomy, showed marked reduction of the tear-drop poikilocytosis and leukoerythroblastosis. We believe that the spleen plays an important role in their formation, but the exact mechanism remains unclear.

Matched MeSH terms: Hemoglobins
Fulltext Reduced red blood cell deformability in Plasmodium knowlesi malaria

Barber BE, Russell B, Grigg MJ, Zhang R, William T, Amir A, et al.

Blood Adv, 2018 02 27;2(4):433-443.
PMID: 29487058 DOI: 10.1182/bloodadvances.2017013730

The simian parasite Plasmodium knowlesi can cause severe and fatal human malaria. However, little is known about the pathogenesis of this disease. In falciparum malaria, reduced red blood cell deformability (RBC-D) contributes to microvascular obstruction and impaired organ perfusion. In P knowlesi infection, impaired microcirculatory flow has been observed in Macaca mulatta (rhesus macaques), unnatural hosts who develop severe and fatal disease. However, RBC-D has not been measured in human infection or in the natural host M fascicularis (long-tailed macaques). Using ektacytometry, we measured RBC-D in adults with severe and non-severe knowlesi and falciparum malaria and in healthy controls. In addition, we used micropipette aspiration to determine the relative stiffness of infected RBCs (iRBCs) and uninfected RBCs (uRBCs) in P knowlesi-infected humans and M fascicularis Ektacytometry demonstrated that RBC-D overall was reduced in human knowlesi malaria in proportion to disease severity, and in severe knowlesi malaria, it was comparable to that of severe falciparum malaria. RBC-D correlated inversely with parasitemia and lactate in knowlesi malaria and HRP2 in falciparum malaria, and it correlated with hemoglobin nadir in knowlesi malaria. Micropipette aspiration confirmed that in humans, P knowlesi infection increased stiffness of both iRBCs and uRBCs, with the latter mostly the result of echinocytosis. In contrast, in the natural host M fascicularis, echinocyte formation was not observed, and the RBC-D of uRBCs was unaffected. In unnatural primate hosts of P knowlesi, including humans, reduced deformability of iRBCs and uRBCs may represent a key pathogenic mechanism leading to microvascular accumulation, impaired organ perfusion, and anemia.

Matched MeSH terms: Hemoglobins
Red cell metabolism and severe neonatal jaundice in West Malaysia

Lie-Injo LE, Virik HK, Lim PW, Lie AK, Ganesan J

Acta Haematol., 1977;58(3):152-60.
PMID: 409030 DOI: 10.1159/000207822

A study was carried out of 332 babies suffering from severe neonatal jaundice who were admitted to the General Hospital, Kuala Lumpar, Malaysia. Of the 332 neonates, 51 were premature and 281 were full-term babies, 178 (110 Chinese, 58 Malay, 9 Indian and 1 European-Pakistani) had bilirubin levels of 20 mg% or higher, requiring exchange blood transfusion. Of the Chinese neonates, 23 (20.9%) had G6PD deficiency, 9 (8.2%) had Hb Bart's and 2 (1.8%) had an abnormal haemoglobin, one Hb Q and one fetal variant. Among the Malay infants, 10 (17.2%) had G6PD deficiency, 7 (12.1%) had Hb Bart's and 10 (17.2%) had abnormal haemoglobins (four had Hb E trait, one had Hb K and Bart's in addition to Hb E, three had Hb CoSp with Hb Bart's, one had Hb Q and one Hb Tak). One of the nine Indian neonates had G6PD deficiency and one had Hb S trait. The one European-Pakistani baby was a carrier of Hb D Punjab. In addition to G6PD deficiency, abnormal haemoglobins seem to have contributed to the high incidence of severe neonatal jaundice in Malaysia. The mean activities of GP, GR and GR after stimulation with FAD were higher, while the mean activity of PK and mean level of reduced glutathione were lower than in normal cord bloods. The percent increase of GR after FAD stimulation was significantly lower; fewer in this group had increases above 20% than in normal cord blood. The possible significance of the findings is discussed.

Matched MeSH terms: Hemoglobins, Abnormal/analysis

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