Monozygotic Twins with Sickle Cell Anemia and Discordant Clinical Courses: Clinical and Laboratory Studies
作者:
AminR. R.,
BauersachsR. M.,
MeiszlmanH. J.,
HlohandasN.,
HebbelR. P.,
BowenP. E.,
SchlegelR. A.,
WilliamsonP.,
WestermanM. P.,
期刊:
Hemoglobin
(Taylor Available online 1991)
卷期:
Volume 15,
issue 4
页码: 247-256
ISSN:0363-0269
年代: 1991
DOI:10.3109/03630269109027877
出版商: Taylor&Francis
数据来源: Taylor
摘要:
We describe a rare set of monozygotic twins with coexistent sickle cell anemia andα-/ααthalassemia who have asynchronous painful crises of different frequency and severity. Studies include measurements of cell deformability and other hemorheologic tests, cell density distribution, the percentage of irreversibly sickled cells, adherence of red cells to endothelial cells, membrane he me and membrane free iron, calcium containing internal vesicles and serum antioxidants. Results of these studies, including estimates of organ damage (bone, spleen, retina), were similar except for an increase in red cell membrane free iron in the patient with more frequent and severe painful crises. The study supports the concept that non-inherited factors are important contributors to the frequcncy and severity of painful crises in sickle cell anemia.
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