Report of three Bengali cases with Hemoglobin E variant in Najran

Four hundred and fifteen specimens of cord blood collected from the Thai population were examined; 22 contained an electrophoretically "fast"-moving component of hemoglobin, an incidence of 5.2%. No other abnormal hemoglobin was found in the specimens of cord blood. Eleven of the babies with abnormal hemoglobin were examined; hematologic findings were within normal limits. Five were followed for 2½ to 8 months; the abnormal component disappeared within 76 to 101 days. One of these children subsequently developed hemoglobin E. Another baby, 2 days of age when first seen, had the "fast" component of hemoglobin in the blood. She was the only child in the group who had marked hepatomegaly, splenomegaly, reticulocytosis, and nucleated erythrocytes in the peripheral blood. These abnormalities subsequently disappeared, but at 112 days a small amount of the "fast" component of hemoglobin persisted and hemoglobin E had appeared. The blood of 412 mothers revealed an incident of 10% hemoglobin E, the only abnormal hemoglobin detected by paper electrophoresis. The concentration of hemoglobin A2 was determined on the blood drawn from nine parents of babies with abnormal "fast" hemoglobin; the value was normal in all instances. Microcytosis and increased resistance of the erythrocytes in hypotonic saline solution, however, was found frequently among the parents of affected babies. The abnormal "fast" component of hemoglobin was identical with "Barts" hemoglobin. Its characteristics suggest that it may be an abnormal fetal hemoglobin.

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Pattern of Thalassemia and Hemoglobinopathies Among Anemic Under-Five Children of Northern Bangladesh: A Hospital Based Cross Sectional Study

10.21203/rs.3.rs-157764/v1 ◽

2021 ◽

Author(s):

Md. Abdur Rafi ◽

M. Morsed Zaman ◽

Md. Golam Hossain

Keyword(s):

Cross Sectional Study ◽

Severe Form ◽

Health Concern ◽

Sectional Study ◽

Cross Sectional ◽

Hemoglobin E ◽

Under Five ◽

Under Five Children ◽

Thalassemia Minor ◽

Different Types

Abstract Background Congenital hemoglobin defects are significant global health concern affecting more than 330,000 newborns every year. In Bangladesh more than half of the under-five children are anemic, but the contribution of hemoglobin disorders to childhood anemia is quite unexplored. Hence, the objective of the present study was to investigate the prevalence and pattern of hemoglobinopathies among anemic under-five (age 6–59 months) children visiting the hematology department of Rajshahi Medical College Hospital (RMCH). Methods This was a retrospective cross-sectional study among anemic patients aged between 6 to 59 months conducted in the hematology department of RMCH from July 2018 to June 2020. The guideline of WHO was used to define the severity of anemia. Hemoglobinopathies was measured using standard methods. Chi-square test was used to find the association between two categorical factors. Results Female and younger children included in the study were more likely to suffer from moderate to severe form of anemia compared to their counterparts. Out of 534 patients 183 were suffering from different types of hemoglobinopathies (prevalence 34.3%). E-β-Thalassemia was the most common type of hemoglobinopathy (14%) followed by Hemoglobin E trait (11%) and β-Thalassemia minor (5.6%). Prevalence of these hemoglobin disorders was comparatively higher among female and older children (p < 0.05). Patients with E-β-Thalassemia and β-Thalassemia major of our study were mostly suffering from severe form of anemia while patients with hemoglobin E trait and β-Thalassemia minor were mostly suffering from mild anemia (p < 0.01). Conclusions A remarkable number of children were suffering from severe anemia and different types of hemoglobinopathies. Gender and age group were the risk factors of anemia and hemoglobinopathies among under-five children. Health authorities of Bangladesh should especially take care of anemic children in this country.

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Efficacy evaluation of new hemoglobin E screening test in community hospitals

Scientific Research and Essays ◽

10.5897/sre2014.5896 ◽

2014 ◽

Vol 9 (12) ◽

pp. 528-534

Author(s):

Sudjaroen Yuttana

Keyword(s):

Screening Test ◽

Community Hospitals ◽

Efficacy Evaluation ◽

Hemoglobin E

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Benefits of chronic blood transfusion in hemoglobin E/β thalassemia with pulmonary arterial hypertension

International Journal of General Medicine ◽

10.2147/ijgm.s66610 ◽

2014 ◽

pp. 411 ◽

Cited By ~ 7

Author(s):

Nonlawan Chueamuangphan ◽

Jayanton Patumanond ◽

Wattana Wongtheptien ◽

Weerasak Nawarawong ◽

Apichard Sukornthasarn ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Blood Transfusion ◽

Arterial Hypertension ◽

Beta Thalassemia ◽

Hemoglobin E ◽

Pulmonary Arterial

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IS HEMOGLOBIN E GENE WIDELY SPREAD IN THE STATE OF MADHYA PRADESH IN CENTRAL INDIA? EVIDENCE FROM FIVE TYPICAL FAMILIES

Mediterranean Journal of Hematology and Infectious Diseases ◽

10.4084/mjhid.2014.060 ◽

2014 ◽

Vol 6 (1) ◽

pp. e2014060 ◽

Cited By ~ 1

Author(s):

R S Balgir

Keyword(s):

Sickle Cell ◽

Clinical Manifestations ◽

Central India ◽

Madhya Pradesh ◽

Red Cell ◽

Hemoglobin E ◽

Vulnerable People ◽

Hb E ◽

Double Heterozygosity ◽

First Time

Background: Red cell inherited hemoglobin anomalies are commonly encountered in the central region of India. These cause a public health concern due to high degree of morbidity, mortality, and fetal loss in the backward, underprivileged, and vulnerable people. Purpose: To report five typical families of hemoglobin E disorders identified for the first time in the state of Madhya Pradesh from central India. Methods: Out of a total of 445 couples/families (excluding the present study) with 1526 persons (848 males and 678 females) referred from a tertiary hospital in central India for investigations of anemia/hemoglobinopathies during the period from March 2010 to February 2014, we came across five typical rare couples/families of hemoglobin E disorders worthy of detailed investigations. Laboratory investigations were carried out following the standard procedures after cross checking for quality control from time to time. Results: For the first time, we have encountered nine cases of heterozygous hemoglobin E trait, two members with hemoglobin E-β-thalassemia (double heterozygosity), two cases of sickle cell-hemoglobin E disease (double heterozygosity), and none with homozygous hemoglobin E. Cases of hemoglobin E trait, hemoglobin E-β-thalassemia, sickle cell-β-thalassemia and sickle cell-E disease showed moderate to severe anemia, and target cells, and reduced values of red cell indices like RBC, Hb level, HCT, MCV, MCH and MCHC, representing abnormal hematological profile and clinical manifestations before blood transfusion. Conclusions: Double heterozygosity for hemoglobinopathies such as occurrence of β-thalassemia mutation with structurally abnormal hemoglobins (Hb S and Hb E) is a rare entity, but occurs with severe clinical manifestations only in those areas or communities where these are highly prevalent, testifying the migrations and genetic admixture. Distribution of hemoglobin E and β-thalassemia in different districts of Madhya Pradesh indicates that abnormal Hb E gene has wide spread and needs prevention for the rehabilitation of vulnerable people in central India.

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