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DIAGNOSTIC CHALLENGES IN A POPULATION WITH INCREASED IMMIGRATION: HEMOGLOBINOPATHIES IN THE NEW CENTURY
Author(s):
Joud Zanabili Al-Sibai
,
Joud Zanabili Al-Sibai
Affiliations:
Hematology,HUCA,Oviedo,Spain;Hematology,HUCA,Oviedo,Spain
Ariana Fonseca Mourelle
,
Ariana Fonseca Mourelle
Affiliations:
Hematology,HUCA,Oviedo,Spain
Tamara Arias Fernández
,
Tamara Arias Fernández
Affiliations:
Hematology,HUCA,Oviedo,Spain
Lucia Rita Morais Bras
,
Lucia Rita Morais Bras
Affiliations:
Hematology,HUCA,Oviedo,Spain
Laura Francisca Ávila Idrovo
,
Laura Francisca Ávila Idrovo
Affiliations:
Hematology,HUCA,Oviedo,Spain
Christelle Castañón Fernández
,
Christelle Castañón Fernández
Affiliations:
Hematology,HUCA,Oviedo,Spain
Antonio Solé Magdalena
,
Antonio Solé Magdalena
Affiliations:
Hematology,HUCA,Oviedo,Spain
Angel Bernardo Gutiérrez
,
Angel Bernardo Gutiérrez
Affiliations:
Hematology,HUCA,Oviedo,Spain
Regina Llorente de Jesus
Regina Llorente de Jesus
Affiliations:
Hematology,HUCA,Oviedo,Spain
(Abstract release date: 05/18/17) EHA Library. J. 05/18/17; 182868; PB2155
Mrs. Joud
Mrs. Joud
Contributions
PDF Abstract
Abstract

Abstract: PB2155

Type: Publication Only

Background
The diagnosis of hemoglobinopathies (Hbpts) has changed in recent years due to immigration, with an increase in structural Hbpts. In our region, Asturias, population census is 1,061.756 habitants; 48,097 out of them are immigrants.

Aims
Review the incidence of structural Hbpts and thalassemias in our region in the last 10 years.

Methods
A retrospective analysis was performed with 1202 hemoglobin (Hb) studies at Hospital Universitario Central de Asturias between January 2006 and March 2016. The studies came from medical applications, the finding of abnormal Hb patterns in the HbA1c test or suggestive results of thalassemia on hematimetry test.

Studies were performed by high-performance liquid chromatography (HPLC) with the Variant Hemoglobin Testing machine of Bio Rad, and Hb electrophoresis with Paragon plates until May 2013. Since then, it has been performed by capillary electrophoresis (CE) with the MINICAP System of the Sebia laboratory.

Results
We analyzed 1202 patients, 49% were males and the median age was 33 years (range 0-85).

We found 562 patologic studies.
- Thalassemia were detected in 390; 337 were β or δβ (86.4%); 54% came from Spain. The cases of β-thalassemia were: 5 intermedia, 3 major, 1 δβ-homozygote and the remainder were minor (97%). All thalassemia major, 3 intermediate and 74%of minor were Caucasian. Anemia was found in all of major thalassemia (median Hb 6.2g / dL, range 6-6.5), in 3 of intermedia (11.4g / dL, range 6-10), and in 197 of minor (10.87g / dL, range 6.9-11.9).
- Structural Hbpts were found in 170, the predominant was Hb S (n = 125). Only 17 sickle cells (Hb SS and Hb SC). Most of them black (n = 14) coming from Africa (n = 10) and South America (n = 5). Eighty six percent (n = 108) were heterozygous (Hb SA), mainly from Africa (n = 56) and South America (n = 23). Anemia were seen: 4 Hb SC (median Hb 10.5g / dL, range: 9.4-11.2), 9 Hb SS (7.94g / dL, range 5.2-9.7) and 37 heterozygotes (10.15g / dL, range 6.7-11.9).
There are two peaks of higher incidence of structural Hbpts, in 2008 coinciding with the creation of the Tropical Diseases Unit and since 2013 when detection increases with the introduction of HbA1c test. The increase in thalassemias was due to the decision to extend studies due to pathological findings in hematimetry results.

Conclusion

In our area there is a predominance of β-thalassemia minor.
Structural Hbpts are the main diagnosis in immigrants. The incidence is still small, although increasing in the last 3 years, so a neonatal screening program is being implemented.
Both HPLC and EC are simple, fast and efficient methods in the diagnosis of Hbpts.
In our area there is a predominance of β-thalassemia minor.
Structural Hbpts are the main diagnosis in immigrants. The incidence is still small, although increasing in the last 3 years, so a neonatal screening program is being implemented.
Both HPLC and CE are simple, fast and efficient methods in the diagnosis of Hbpts.

Session topic: 25. Sickle cell disease

Keyword(s): Thalassemia, sickle cell disease

Abstract: PB2155

Type: Publication Only

Background
The diagnosis of hemoglobinopathies (Hbpts) has changed in recent years due to immigration, with an increase in structural Hbpts. In our region, Asturias, population census is 1,061.756 habitants; 48,097 out of them are immigrants.

Aims
Review the incidence of structural Hbpts and thalassemias in our region in the last 10 years.

Methods
A retrospective analysis was performed with 1202 hemoglobin (Hb) studies at Hospital Universitario Central de Asturias between January 2006 and March 2016. The studies came from medical applications, the finding of abnormal Hb patterns in the HbA1c test or suggestive results of thalassemia on hematimetry test.

Studies were performed by high-performance liquid chromatography (HPLC) with the Variant Hemoglobin Testing machine of Bio Rad, and Hb electrophoresis with Paragon plates until May 2013. Since then, it has been performed by capillary electrophoresis (CE) with the MINICAP System of the Sebia laboratory.

Results
We analyzed 1202 patients, 49% were males and the median age was 33 years (range 0-85).

We found 562 patologic studies.
- Thalassemia were detected in 390; 337 were β or δβ (86.4%); 54% came from Spain. The cases of β-thalassemia were: 5 intermedia, 3 major, 1 δβ-homozygote and the remainder were minor (97%). All thalassemia major, 3 intermediate and 74%of minor were Caucasian. Anemia was found in all of major thalassemia (median Hb 6.2g / dL, range 6-6.5), in 3 of intermedia (11.4g / dL, range 6-10), and in 197 of minor (10.87g / dL, range 6.9-11.9).
- Structural Hbpts were found in 170, the predominant was Hb S (n = 125). Only 17 sickle cells (Hb SS and Hb SC). Most of them black (n = 14) coming from Africa (n = 10) and South America (n = 5). Eighty six percent (n = 108) were heterozygous (Hb SA), mainly from Africa (n = 56) and South America (n = 23). Anemia were seen: 4 Hb SC (median Hb 10.5g / dL, range: 9.4-11.2), 9 Hb SS (7.94g / dL, range 5.2-9.7) and 37 heterozygotes (10.15g / dL, range 6.7-11.9).
There are two peaks of higher incidence of structural Hbpts, in 2008 coinciding with the creation of the Tropical Diseases Unit and since 2013 when detection increases with the introduction of HbA1c test. The increase in thalassemias was due to the decision to extend studies due to pathological findings in hematimetry results.

Conclusion

In our area there is a predominance of β-thalassemia minor.
Structural Hbpts are the main diagnosis in immigrants. The incidence is still small, although increasing in the last 3 years, so a neonatal screening program is being implemented.
Both HPLC and EC are simple, fast and efficient methods in the diagnosis of Hbpts.
In our area there is a predominance of β-thalassemia minor.
Structural Hbpts are the main diagnosis in immigrants. The incidence is still small, although increasing in the last 3 years, so a neonatal screening program is being implemented.
Both HPLC and CE are simple, fast and efficient methods in the diagnosis of Hbpts.

Session topic: 25. Sickle cell disease

Keyword(s): Thalassemia, sickle cell disease

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