Sickle cell disease: a chronic inflammatory condition

Homozygous sickle cell disease (SCD) has a wide spectrum of clinical manifestations which varies from an almost asymptomatic condition to severe illness, despite the fact that all subjects with this disease have the same base change in their DNA. The source of this variation is partly environmental,...

Full description

Saved in:
Bibliographic Details
Published in:Medical hypotheses 2001-07, Vol.57 (1), p.46-50
Main Authors: Chies, J.A.B., Nardi, N.B.
Format: Article
Language:English
Subjects:
Anemia, Sickle Cell - drug therapy
Anemia, Sickle Cell - immunology
Anemia, Sickle Cell - pathology
Anemias. Hemoglobinopathies
Animals
Biological and medical sciences
Chronic Disease
Diseases of red blood cells
Hematologic and hematopoietic diseases
Humans
Hydroxyurea - therapeutic use
Inflammation - immunology
Inflammation - pathology
Medical sciences
Mice
Mice, Transgenic
Th1 Cells - immunology
Th2 Cells - immunology
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Homozygous sickle cell disease (SCD) has a wide spectrum of clinical manifestations which varies from an almost asymptomatic condition to severe illness, despite the fact that all subjects with this disease have the same base change in their DNA. The source of this variation is partly environmental, but a large part of this variability can derive from the presence of genetic modulators which are not fully understood. It was postulated that some degree of immunodeficiency should be associated with this condition, but no deficiency, directly related to a given component of the immune system, was observed that could explain the high levels of recurrent infections presented by sickle cell disease patients. Reviewing data from the literature we suggest that the influence of the immune system in the variation of clinical manifestations presented by SCD patients is not related with any immunodeficiency but is rather the result of a chronic inflammatory condition.
ISSN:0306-9877
1532-2777
DOI:10.1054/mehy.2000.1310