Irreversibly sickled cells and red cell survival in sickle cell anemia. A study with both DF32P and 51Cr

1978 ◽  
Vol 64 (2) ◽  
pp. A88
PEDIATRICS ◽  
1971 ◽  
Vol 48 (3) ◽  
pp. 457-458
Author(s):  
Annemarie Sommer ◽  
Stella B. Kontras

The incidence of splenomegaly in sickle cell anemia (defined as a spleen easily palpated below the costal margin in quiet respiration) appears to be around 10% after 10 years of age. Persistent splenomegaly in older children is rare and frequently is associated with hypersplenism. Splenectomy has been the treatment of choice in several reported cases based on red cell survival studies before and after splenectomy.1-3 Removal of the spleen has been found to be associated with marked improvement of previously very shortened red cell survival. We want to report the case of a 12-year-old boy with sickle cell anemia, splenomegaly, and sickle cell heart disease who was treated by radiation therapy for his enlarged spleen because of hypersplenism.


Blood ◽  
1972 ◽  
Vol 40 (5) ◽  
pp. 733-739 ◽  
Author(s):  
Blanche P. Alter ◽  
Yuet Wai Kan ◽  
David G. Nathan

Abstract Cyanate prevents sickling in vitro and apparently prolongs the survival of 51Cr-tagged sickle erythrocytes in vivo. Cautious interpretation is required because the effects of cyanate on 51Cr binding to sickle and fetal hemoglobin-containing red cells are unknown, and comparison of the effect of cyanate on sickle red cell survival to control red cell survival must be performed sequentially. We have studied the survival of sickle reticulocytes utilizing radioactive amino acids that are incorporated into hemoglobin. Two informed adult patients with sickle cell disease were studied. In each study, two 50-ml samples of blood were incubated separately with 14C- and 3H-leucine for 2 hr, after which 50 mM cyanate was added to one aliquot for 1 hr. The cells were then washed and reinfused. Frequent venous samples were obtained, and the specific activities of 14C and 3H in the hemoglobin were followed. The t ½ of the carbamylated cells was tripled, but remained below normal. This method provides a generally useful measurement of the influence of drugs bound to red cells on reticulocyte lifespan. The labels are incorporated into the hemoglobin molecule of the reticulocyte, and simultaneous comparison of the survivals of the same cohort of drug-treated and control cells is achieved.


Blood ◽  
2001 ◽  
Vol 98 (5) ◽  
pp. 1577-1584 ◽  
Author(s):  
Kitty de Jong ◽  
Renee K. Emerson ◽  
James Butler ◽  
Jacob Bastacky ◽  
Narla Mohandas ◽  
...  

Several transgenic murine models for sickle cell anemia have been developed that closely reproduce the biochemical and physiological disorders in the human disease. A comprehensive characterization is described of hematologic parameters of mature red blood cells, reticulocytes, and red cell precursors in the bone marrow and spleen of a murine sickle cell model in which erythroid cells expressed exclusively human α, γ, and βS globin. Red cell survival was dramatically decreased in these anemic animals, partially compensated by considerable enhancement in erythropoietic activity. As in humans, these murine sickle cells contain a subpopulation of phosphatidylserine-exposing cells that may play a role in their premature removal. Continuous in vivo generation of this phosphatidylserine-exposing subset may have a significant impact on the pathophysiology of sickle cell disease.


1972 ◽  
Vol 264 (4) ◽  
pp. 283-287 ◽  
Author(s):  
THOMAS A. BENSINGER ◽  
LAVIZA MAHMOOD ◽  
MARCEL E. CONRAD ◽  
PAUL R. McCURDY

Blood ◽  
1975 ◽  
Vol 46 (2) ◽  
pp. 253-259 ◽  
Author(s):  
E Beutler

Abstract Carbon monoxide at a concentration of 1000–2000 ppm was administered to sickle cell disease patients. In each of two patients, one 51Cr red cell survival study was carried out before CO administration, and a second study was initiated a few days before CO administration was started. In both, significant prolongation of red cell survival was observed, suggesting that the rheologic properties of sickle cells were favorably influenced in vivo. The administration of carbon monoxide is not recommended as a treatment for sickle cell disease. However, further trials would seem to be justified if conducted under carefully controlled conditions.


Blood ◽  
1975 ◽  
Vol 45 (2) ◽  
pp. 273-279 ◽  
Author(s):  
PR McCurdy ◽  
L Mahmood ◽  
AS Sherman

Red cell survival was measured in ten subjects with S-C disease and one with S-O Arab (alpha 2 beta 2–121 glu yields lys) disease using both DF32p and 51Cr as tags. Red cell volume was slightly reduced in most patients (87% plus or minus 20% of predicted normal). In nine SC patients, mean red cell life (DF32p) was 28.9 plus or minus 4.0 days. For one SC subject it was significantly longer (47.9 days), as it was for the one with S-O Arab. The S-O Arab subject had irreversibly sickled cells in the peripheral blood, shereas those with SC had few (less than 1/1000 red cells) or none. The S-O Arab hemolysate gelled at a hemmoglobin concentration (16.2 g/100ml) near that for sickle cell anemia hemolysates (15.9 plus or minus 1.0 g/100 ml; n equals 8) but significantly lower than that for SC hemolysates (21.6 plus or minus 1.9 g/100 ml; n equals 5). It seems likely that properties of S-C red cells other than their relative ease of sickling contribute significantly to their rate of hemolysis.


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