Hemoglobin J Mexico has been found in five generations of a large Algerian family. Nine subjects have 55% Hb J although their parents, siblings and offspring may have 31%, the usual quantity found in heterozygotes. Those with 55% Hb J are considered to be homozygous for a chromosome carrying both a normal alpha chain locus and a locus for alphaJ. The proportion of the abnormal hemoglobin in all the subjects is in favor of an unequal expression of both loci, the amount of protein synthesis directed by the alpha J gene being greater than that directed by the alpha A. In two heterozygotes a slightly higher proportion of the Hb J (38%) suggests the presence of a single normal alpha chain locus in trans. An associated alpha-thalassemia was excluded by biosynthetic studies in this family.

Genes↗

PubMedDate not supplied

[Hemoglobinopathies in West-African immigrant workers in France (author's transl)].

In this paper are brought the results obtained in two Parisian hospitals during a survey of abnormal hemoglobins in 540 immigrant workers coming from Africa, mostly from Mali, Mauritania and Senegal. All the subjects investigated were male and between 20 and 40 years old. The studies were performed following internationally standardized technics. The most frequent abnormalities were: Hb S found in 16.3%, Hb C (6.6%), alpha-thalassemia trait (3.1%) and beta-thalassemia trait (3.1%). Some rare abnormalities were also found: delta-chain variants, hereditary persistance of foetal hemoglobin, Hb Hope and Hb Grady. This work emphasizes the high frequency of the different hemoglobin disorders in this population. The easy diagnostic of electrophoretically detectable variants is compared to the more complex situation of thalassemia leading probably to an under estimation of the percentage.

Adult↗

PubMedDate not supplied

[Influence of alpha-thalassemia on the hematologic expression of homozygote drepanocytosis].

A Madagascan child was followed for an homozygous sickle cell disease from the age of 7 to the age of 9 years. The clinical course of the disease, quite moderate till the age of 7, was then made more severe because of frequent painful crises during the observation period. Genetical and haematological data of the patient and his family showed that the two parents, heterozygote for sickle cell disease, and the homozygote patient, also had alpha-thalassemia. In the propositus patient, the association of the two disorders accounts for the quite moderate anaemia (haemoglobin level between 10 and 12 g/dl) and the persistent microcytosis. So, a microcytosis without iron deficiency and an haemoglobin level above 9 g/dl should suggest such an association.

Anemia, Sickle Cell↗

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