Hemoglobin Le Lamentin in the province of Albacete, Spain: Discovery of 32 cases.
OBJECTIVE
Hemoglobin Le Lamentin (α20(B1)His→Gln) is a ubiquitous variant that has been previously described in a small number of isolated patients. We report the incidental observation of Hb Le Lamentin in a large population from the province of Albacete, in southeastern Spain. Our study investigates possible reasons for the elevated number of carriers of this variant and its implications for the management of diabetes in our region.


METHODS
The subjects are 32 diabetic patients whose hemoglobin displayed an unusual peak while they were being tested for glycated hemoglobin at the laboratory of the University General Hospital of Albacete over a 3-year period. Measurements were made by high performance liquid chromatography using a Variant™ II Turbo Kit-2.0, and subsequently the samples of the 32 patients with anomalous peaks were sent to the Hospital Clínico San Carlos (Madrid, Spain) for molecular characterization of any Hb variants.


RESULTS
Molecular studies revealed 31 out of 32 patients heterozygous for Le Lamentin, and in one of them, Hb City of Hope was associated with Hb Le Lamentin. The remaining patient was homozygous for the Le Lamentin mutation. Additionally, most patients were native to the northeastern half of the province of Albacete and were unrelated.


CONCLUSIONS
Our study describes the largest finding to date of hemoglobin Le Lamentin in a sample of patients. The fact that our region has been perpetually depopulated, with a population that has remained stable in small localities over the centuries, may have favored the survival of the mutation. Since the presence of this variant underestimates the true value of glycated hemoglobin measured by HPLC, it is necessary to systematically review chromatograms.
Servicio de Análisis Clínicos, Hospital General Universitario de Albacete, Albacete, Spain. Electronic address: rosalinaml@hotmail.es.
Rosalina
R
Martínez-López
Servicio de Hematología y Hemoterapia, Hospital Clínico San Carlos, Madrid, Spain.
Paloma
P
Ropero
Servicio de Análisis Clínicos, Hospital General Universitario de Albacete, Albacete, Spain.
Cristina
C
Ballesteros Gallar
Servicio de Análisis Clínicos, Hospital General Universitario de Albacete, Albacete, Spain.
Carlos
C
Romero Román
Servicio de Hematología y Hemoterapia, Hospital Clínico San Carlos, Madrid, Spain.
Félix
F
de la Fuente-Gonzalo
Servicio de Hematología y Hemoterapia, Hospital Clínico San Carlos, Madrid, Spain.
Jorge
J
M Nieto
Servicio de Análisis Clínicos, Hospital General Universitario de Albacete, Albacete, Spain.
Laura
L
Navarro Casado
Servicio de Hematología y Hemoterapia, Hospital Clínico San Carlos, Madrid, Spain.
Fernando A
FA
González
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002851:Chromatography, High Pressure Liquid; D004252:DNA Mutational Analysis; D003920:Diabetes Mellitus / Q000097:blood* / Q000235:genetics; D005260:Female; D006442:Glycated Hemoglobin / Q000378:metabolism; D006455:Hemoglobins, Abnormal / Q000235:genetics / Q000378:metabolism*; D006579:Heterozygote; D006801:Humans; D008297:Male; D008875:Middle Aged; D013030:Spain
D006442:Glycated Hemoglobin; D006455:Hemoglobins, Abnormal; C517652:hemoglobin A1c protein, human; C035852:hemoglobin Le Lamentin
Accuracy; Diabetic patient; Hb Le Lamentin; HbA1c; Largest population; Southeastern Spain