scholarly journals Haemoglobin D Trait-An Uncommon Haemoglobinopathy in Chattogram: A Case Report

2021 ◽  
Vol 20 (1) ◽  
pp. 81-83
Author(s):  
Razia Sultana ◽  
Tani Gosh

This case presents haemoglobin D Trait (Hb-D) which is a rare disease in Bangladesh. It is a heterozygous state for haemoglobin D who came to Laboratory Medicine Department of Chattogram Maa Shishu-O-General Hospital (CMSOGH) for heamoglobin electrophoresis. Our patient was 9 years old girl from Noakhali, Bangladesh. She was clinically silent with no splenomegaly. Her peripheral blood film was microcytic hypochromic anaemia and Capillary Electrophoresis (CE) of Hb revealed Hb D Trait. On family screening her father was Hb E trait, mother was Hb D trait and younger sister turned out normal Hb study. Compound heterozygosity for haemoglobin D and haemoglobin E may be present in subsequent pregnancy of her mother. Chatt Maa Shi Hosp Med Coll J; Vol.20 (1); January 2021; Page 81-83

Author(s):  
Naincy Rastogi ◽  
Arvind S. Bhake

Background: Mild microcytic hypochromic anaemias due to iron deficiency (IDA) and beta thalassemia trait(β-TT) continue to be a cause of significant burden to the society, particularly in the poorer developing countries. The objective of the present study was to study the RBC based indices in patients of marked anisopoikilocytosis in determining the etiology of it, to standardize few automated red cell parameters, and also objective grading of RBC morphology on peripheral smear and interpreting its utility in indicating a diagnosis. Also, to establish a relation between value of RBC indices with that of degree of anisocytosis.Methods: A total of 500 patients diagnosed with mild microcytic hypochromic anaemia on complete blood count and peripheral blood film were included in the study. Hb, RBC count, MCV, MCH and RDW obtained from the electronic cell counter were used to calculate discrimination indices by various mathematical formulae. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and Youden’s index (YI) were calculated.Results: Green and King index demonstrated the lowest sensitivity of 70.51%. Mentzer index demonstrated the highest specificity of 96.80%. The highest and lowest PPV were found for Mentzer index (97.09%) and Sehgal index (92.81%) respectively. Sehgal’s index demonstrated the highest NPV of 95.96% and lowest NPV was exhibited by G and K (87.9%). The highest and the lowest values for Youden’s index were shown by Sehgal’s index (87.82%) and G and K index (68.47%).Conclusions: Sehgal’s index followed by Mentzer index are highly sensitive and reasonably specific in differentiating β-TT from IDA and none of the indices is 100% sensitive and specific. Though HbA2 estimation is the gold standard for diagnosing β-TT, in developing countries, Sehgal index followed by Mentzer et al, index can be used to screen mild microcytic hypochromic anaemia cases to eliminate as many false positive cases as possible to reduce the financial cost.


2011 ◽  
Vol 24 (1) ◽  
pp. 34-37
Author(s):  
MR Khan ◽  
MS Uddin Shah ◽  
MA Aziz ◽  
MMR Khan ◽  
M Khalilur Rahman ◽  
...  

HbE is the most common abnormal hemoglobin of Southeast Asia and is almost limited to this region. It is estimated that 30 million people are heterozygous for HbE and that 1 million are homozygous. Aim of this study was to know the number of homozygous for haemoglobin E condition (Hb E disease) patients among patients suspected of having hereditary haemoglobin disorders referred to the regional laboratory, in Rajshahi. From April 2009 to March 2012, anaemic patients suspected of having hereditary haemoglobin disorder (haemoglobinopathy or thalassemia) were referred to the divisional laboratory, Rajshahi for hemoglobin electrophoresis from the physicians of different areas of Rajshahi division and part of Khulna division. Patient’s age ranging from 01 to 85 yrs of both sexes. Two to three milliliter blood was collected in tubes containing EDTA for complete blood count (CBC) and analysis of hemoglobin variants. CBC was measured by the Erma PC 604 particle counter. Peripheral blood film were examined after staining with Wright’s stain.9 Hemoglobin electrophoresis was carried out on cellulose acetate using TEB buffer, pH 8.6.9. Of 707 anaemic patients suspected of having hereditary haemoglobin disorder (haemoglobinopathy or thalassemia) 333 (47%) were abnormal. Of this abnormal patients 35 (11%) revealed a thalassemia-like disorder, 53 ( 17%) patients revealed haemglobin E disease, 46 ( 14%) patients haemglobin E trait and 188 ( 57%) patients having double heterozygous (Hb E/β thalassemia) disorder. The birth incidence for homozygous babies and or Eβ thalassemia would be increases per year. Population groups with higher frequencies require screening programmes and facilities for antenatal diagnosis. Future comprehensive study are require to know the actual prevalence. TAJ 2011; 24(1): 34-37


1968 ◽  
Vol 21 (6) ◽  
pp. 788-788
Author(s):  
A. G. Signy

2021 ◽  
Vol 8 (14) ◽  
pp. 888-892
Author(s):  
Blessy Mary Thomas ◽  
Sheila Das ◽  
Sunil Antony ◽  
Alice David

BACKGROUND Microcytic hypochromic anaemia is commonly due to iron deficiency, anaemia of chronic disorder [ACD] and thalassaemic syndromes. Reticulocyte count reflects the erythropoietic activity of bone marrow and is thus useful in both diagnosing anaemias and monitoring bone marrow response to therapy METHODS All samples were selected from routine blood counts, and sent for investigation of anaemia, over a period of two years. These samples were run on the DxH800 (Beckman Coulter). 385 cases were selected for the study. Blood analysis for all these cases had been requested by general practitioners to investigate anaemia. These blood samples had been collected in ethylenediaminetetraacetic acid (EDTA) anticoagulant vacutainers and processed within 2 hours of collection. Determination of red cell and reticulocyte parameters in all blood samples, was performed using the Beckman Coulter 7-part analyser [Unicell DxH 800]. RESULTS Of the 156 cases of microcytic hypochromic anaemia studied, iron deficiency anaemia (IDA) was present in 91 cases, anaemia of chronic disorder (ACD) in 50 cases, beta thalassemia trait (BTT) in 15 cases. Of the 50 ACD cases, 37 were associated with IDA. The control group comprised of 229 adult medical students (143 women and 103 men) with a median age of 18.84 ± 0.98 years. We also had 4 cases of other haemoglobinopathies, which were microcytic hypochromic, but were not included in our study as the number of cases was too less to be analysed. CONCLUSIONS New reticulocyte parameters are useful for evaluation of iron status and diagnosing iron deficiency anaemias. They also are reliable parameters for recognising subsets of anaemic patients thereby improving the management of anaemia. KEYWORDS Reticulocyte, Microcytic, Hypochromic, Anaemia, Beckman Coulter


2018 ◽  
Vol 11 (1) ◽  
pp. e227668
Author(s):  
Emily Finan ◽  
Joe Joseph

We report the unusual case of a patient found to have a low glycosylated haemoglobin (HbA1c) despite having recently been diagnosed with diabetes mellitus type 2. The patient, who was not anaemic, with no symptoms or family history of haematological conditions, was subsequently found to have an elevated reticulocyte count, inferring increased red cell turnover as the culprit for the discordant HbA1c result. A diagnosis of hereditary spherocytosis was made based on characteristic peripheral blood film appearances and confirmed by eosin-5-maleimide binding test. Exposure of an undiagnosed haemolytic anaemia by virtue of a low HbA1c is uncommon. However, conditions that distort HbA1c measurements are not infrequent. This case should serve to remind clinicians of the limitations of HbA1c in specified situations, and to remain vigilant when interpreting results.


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