scholarly journals Alpha-thalassemia is related to prolonged survival in sickle cell anemia

Blood ◽  
1983 ◽  
Vol 62 (2) ◽  
pp. 286-290 ◽  
Author(s):  
JG Mears ◽  
HM Lachman ◽  
D Labie ◽  
RL Nagel
Keyword(s):  
Life Expectancy ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Prolonged Survival ◽  
Alpha Thalassemia ◽  
The Usa ◽  
Thalassemia Trait ◽  
Alpha Gene ◽  
Black Populations

Abstract We have determined the frequency of deletional alpha-thalassemia in black populations in the USA and Africa that harbor sickle cell anemia. In normals, the frequency of the chromosome bearing a deletion of one of the two normal alpha gene loci, designated (-alpha), ranged from 0.12 to 0.16, and in sickle trait subjects, the frequency ranged from 0.18 to 0.20. By contrast, in sickle cell anemia subjects, the frequency was significantly greater and ranged from 0.22 to 0.33. Analysis demonstrated that the greater frequency in the last group was primarily a result of an increased number of subjects with alpha- thalassemia trait (also called homozygous alpha-thalassemia-2). In addition, the frequency of the (-alpha) chromosome was found to increase progressively with age, supporting the hypothesis that alpha- thalassemia is favorable to the survival of subjects with sickle cell anemia. Thus, individuals who inherit alpha-thalassemia and sickle cell anemia may represent a subgroup of patients with a longer life expectancy.

scholarly journals Alpha-thalassemia is related to prolonged survival in sickle cell anemia

Blood ◽  
1983 ◽  
Vol 62 (2) ◽  
pp. 286-290 ◽  
Author(s):  
JG Mears ◽  
HM Lachman ◽  
D Labie ◽  
RL Nagel
Keyword(s):  
Life Expectancy ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Prolonged Survival ◽  
Alpha Thalassemia ◽  
The Usa ◽  
Thalassemia Trait ◽  
Alpha Gene ◽  
Black Populations

We have determined the frequency of deletional alpha-thalassemia in black populations in the USA and Africa that harbor sickle cell anemia. In normals, the frequency of the chromosome bearing a deletion of one of the two normal alpha gene loci, designated (-alpha), ranged from 0.12 to 0.16, and in sickle trait subjects, the frequency ranged from 0.18 to 0.20. By contrast, in sickle cell anemia subjects, the frequency was significantly greater and ranged from 0.22 to 0.33. Analysis demonstrated that the greater frequency in the last group was primarily a result of an increased number of subjects with alpha- thalassemia trait (also called homozygous alpha-thalassemia-2). In addition, the frequency of the (-alpha) chromosome was found to increase progressively with age, supporting the hypothesis that alpha- thalassemia is favorable to the survival of subjects with sickle cell anemia. Thus, individuals who inherit alpha-thalassemia and sickle cell anemia may represent a subgroup of patients with a longer life expectancy.

An age dependent response to hydroxyurea in pediatric sickle cell anemia patients with alpha thalassemia trait

2017 ◽  
Vol 66 ◽  
pp. 19-23
Author(s):  
Lisa Figueiredo ◽  
Kerry Morrone ◽  
Catherine Wei ◽  
Karen Ireland ◽  
Hillel W. Cohen ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Alpha Thalassemia ◽  
Age Dependent ◽  
Thalassemia Trait

Alpha thalassemia, but not βS-globin haplotypes, influence sickle cell anemia clinical outcome in a large, single-center Brazilian cohort

2021 ◽  
Author(s):  
Betânia Lucena Domingues Hatzlhofer ◽  
Diego Antonio Pereira-Martins ◽  
Igor de Farias Domingos ◽  
Gabriela da Silva Arcanjo ◽  
Isabel Weinhäuser ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Single Center ◽  
Alpha Thalassemia ◽  
Brazilian Cohort

scholarly journals Sickle Cell Anemia and Babesia Infection

Pathogens ◽  
2021 ◽  
Vol 10 (11) ◽  
pp. 1435
Author(s):  
Divya Beri ◽  
Manpreet Singh ◽  
Marilis Rodriguez ◽  
Karina Yazdanbakhsh ◽  
Cheryl Ann Lobo
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Current Knowledge ◽  
Globin Gene ◽  
Environmental Niche ◽  
Parasite Resistance ◽  
The Usa ◽  
Human Infections

Babesia is an intraerythrocytic, obligate Apicomplexan parasite that has, in the last century, been implicated in human infections via zoonosis and is now widespread, especially in parts of the USA and Europe. It is naturally transmitted by the bite of a tick, but transfused blood from infected donors has also proven to be a major source of transmission. When infected, most humans are clinically asymptomatic, but the parasite can prove to be lethal when it infects immunocompromised individuals. Hemolysis and anemia are two common symptoms that accompany many infectious diseases, and this is particularly true of parasitic diseases that target red cells. Clinically, this becomes an acute problem for subjects who are prone to hemolysis and depend on frequent transfusions, like patients with sickle cell anemia or thalassemia. Little is known about Babesia’s pathogenesis in these hemoglobinopathies, and most parallels are drawn from its evolutionarily related Plasmodium parasite which shares the same environmental niche, the RBCs, in the human host. In vitro as well as in vivo Babesia-infected mouse sickle cell disease (SCD) models support the inhibition of intra-erythrocytic parasite proliferation, but mechanisms driving the protection of such hemoglobinopathies against infection are not fully studied. This review provides an overview of our current knowledge of Babesia infection and hemoglobinopathies, focusing on possible mechanisms behind this parasite resistance and the clinical repercussions faced by Babesia-infected human hosts harboring mutations in their globin gene.

scholarly journals The leftward deletion alpha-thal-2 haplotype in a black subject with hemoglobin SS

Blood ◽  
1985 ◽  
Vol 65 (3) ◽  
pp. 769-771
Author(s):  
SH Embury ◽  
MA Gholson ◽  
P Gillette ◽  
RF Rieder
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Black Population ◽  
Alpha Thalassemia ◽  
Black Individual ◽  
Black Subject ◽  
The Common ◽  
Cellular Pathology

We have identified a black individual with homozygous sickle cell anemia who is the silent carrier of alpha-thalassemia (genotype - alpha/alpha alpha) due to heterozygosity for the leftward deletion alpha-thal-2 haplotype. This deletion has not been described previously in a black subject and is the only leftward deletion that we have found among 255 alpha-thal-2 chromosomes from sickle cell subjects. Its effects on the clinical, hematologic, biosynthetic, and cellular pathology of sickle cell anemia resemble those reported for the common alpha-thalassemia genotypes of the black population.

scholarly journals Age dependence of the gene frequency of alpha-thalassemia in sickle cell anemia in Cuba [letter]

Blood ◽  
1996 ◽  
Vol 88 (5) ◽  
pp. 1898-1899
Author(s):  
G Martinez ◽  
A Muniz ◽  
E Svarch ◽  
E Espinosa ◽  
RL Nagel
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Gene Frequency ◽  
Age Dependence ◽  
Alpha Thalassemia

Alpha thalassemia and stroke risk in sickle cell anemia

1994 ◽  
Vol 45 (4) ◽  
pp. 279-282 ◽  
Author(s):  
Robert J. Adams ◽  
Abdullah Kutlar ◽  
Virgil McKie ◽  
Elizabeth Carl ◽  
Fenwick T. Nichols ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Stroke Risk ◽  
Alpha Thalassemia

Alpha thalassemia protects sickle cell anemia patients from macro-albuminuria through its effects on red blood cell rheological properties

2014 ◽  
Vol 57 (1) ◽  
pp. 63-72 ◽  
Author(s):  
Yann Lamarre ◽  
Marc Romana ◽  
Nathalie Lemonne ◽  
Marie-Dominique Hardy-Dessources ◽  
Vanessa Tarer ◽  
...  
Keyword(s):  
Blood Cell ◽  
Rheological Properties ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Blood Cell ◽  
Alpha Thalassemia

scholarly journals The Senegal DNA haplotype is associated with the amelioration of anemia in African-American sickle cell anemia patients

Blood ◽  
1991 ◽  
Vol 77 (6) ◽  
pp. 1371-1375 ◽  
Author(s):  
RL Nagel ◽  
S Erlingsson ◽  
ME Fabry ◽  
H Croizat ◽  
SM Susuka ◽  
...  
Keyword(s):  
New York ◽  
Gene Cluster ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Globin Gene ◽  
Inhibitory Effect ◽  
Hemoglobin F ◽  
Globin Gene Cluster ◽  
Alpha Gene ◽  
Hbf Level

Abstract We have previously determined that in African sickle cell anemia (SS) patients three different beta-like globin gene cluster haplotypes are associated with different percent G gamma (one of the two types of non- alpha chains comprising hemoglobin F [HbF]), mean percent HbF, and percent dense cells. We report now that in adult New York SS patients, the presence of at least one chromosome with the Senegal haplotype is associated with higher Hb levels (1.2 g/dL higher) than is found for any other non-Senegal haplotype (P less than .004). The percent reticulocytes and the serum bilirubin levels were lower in these patients. When the effect of alpha-gene number was analyzed by examining a sample of SS patients with concomitant alpha-thalassemia, the same results were obtained. Because the HbF level is significantly higher among the Senegal haplotype carriers in this sample, the inhibitory effect on sickling of this Hb variant may be one of the reasons for the haplotype effect. We conclude that the Senegal beta- like globin gene cluster haplotype is associated with an amelioration of the hemolytic anemia that characterizes sickle cell disease.

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