scholarly journals Red cell life span in sickle cell-hemoglobin C disease with a note about sickle cell-hemoglobin O ARAB

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.

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

Abstract 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.


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.


1957 ◽  
Vol 35 (1) ◽  
pp. 1171-1181
Author(s):  
L. G. Israels ◽  
A. Chutorian ◽  
G. E. Delory ◽  
Esther Israels

Sulphaemoglobinaemia was produced in rabbits by the injection of para-aminopropriophenone and calcium sulphide. The disappearance of this pigment from the blood was used as an index of red cell survival. Sulphaemoglobin disappeared in an exponential fashion, indicating a mean red cell life span of 36 days. The red cells were also tagged with Cr51, and this method of measuring erythrocyte life span yielded values strongly suggesting that sulphaemoglobin in the red cell impairs its viability and leads to random cell destruction. Under these conditions it would seem that the disappearance rate of sulphaemoglobin is not a true measure of red cell survival.


1960 ◽  
Vol 198 (1) ◽  
pp. 183-186 ◽  
Author(s):  
S. M. Weissman ◽  
T. A. Waldmann ◽  
N. I. Berlin

The quantitative measurement of erythropoiesis requires the simultaneous determination of total red cell volume, rate of production of red cells and the red cell life span. The total red cell volume was measured with autologous Cr51-labeled red cells, the rate of production of red cells from the rate of disappearance of radioiron from the plasma and uptake by red cells, the red cell life span with C14-labeled glycine and the apparent red cell survival T1/2 with Cr51. The average total red cell volume of the dogs studied was 38.6 cc/kg; the plasma radioiron T1/2 was 66 minutes; the red cell radio-iron uptake was 80%; the serum iron was 102 µg/100 cc, and the plasma volume calculated from the peripheral hematocrit and total red cell volume was 46 cc/kg, and from the extrapolation to t0 of the radioiron disappearance was 48 cc/kg. From these figures the plasma iron turnover was calculated to be 0.63 mg/kg/day and the red cell iron renewal rate 1.26%/day. The average red cell life span was 108 days; the average apparent T1/2 of Cr51 red cell survival was 24.3 days; the average elution rate of Cr51 was 1.77%/day.


Blood ◽  
1976 ◽  
Vol 47 (3) ◽  
pp. 481-488 ◽  
Author(s):  
S Charache ◽  
R Dreyer ◽  
I Zimmerman ◽  
CK Hsu

Abstract Nitrogen mustard and nor-nitrogen mustard inhibit sickling, but the concentrations required would be associated with unacceptable toxicity if these agents were administered to patients. Red cells could be treated extracorporeally and infused back into donors, if the alkylating agent could be removed or inactivated, if the treatment per se did not significantly shorten red cell survival, and if viable alkylated lymphocytes could be eliminated from the treated blood. To estimate whether these conditions could be met in a clinical trial, red cells from four dogs were alkylated at 6-wk intervals. No toxic reactions were observed, although not all nor-nitrogen mustard was removed by the washing procedure. Red cell survival was shortened to about half that of control cells, using concentrations of alkylating agent which reduce sickling by 50%. Lymphocytes from treated blood could still exclude trypan blue, but could not be shown to circulate after reinfusion into donor dogs. If alkylating agents are used to treat patients' cells, inhibition of sickling may outweigh the shortening of red cell life span induced by the treatment; blood should probably be irradiated before infusion to avoid administration of alkylated and potentially mutated, but viable, lymphocytes.


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 ◽  
1976 ◽  
Vol 47 (3) ◽  
pp. 481-488
Author(s):  
S Charache ◽  
R Dreyer ◽  
I Zimmerman ◽  
CK Hsu

Nitrogen mustard and nor-nitrogen mustard inhibit sickling, but the concentrations required would be associated with unacceptable toxicity if these agents were administered to patients. Red cells could be treated extracorporeally and infused back into donors, if the alkylating agent could be removed or inactivated, if the treatment per se did not significantly shorten red cell survival, and if viable alkylated lymphocytes could be eliminated from the treated blood. To estimate whether these conditions could be met in a clinical trial, red cells from four dogs were alkylated at 6-wk intervals. No toxic reactions were observed, although not all nor-nitrogen mustard was removed by the washing procedure. Red cell survival was shortened to about half that of control cells, using concentrations of alkylating agent which reduce sickling by 50%. Lymphocytes from treated blood could still exclude trypan blue, but could not be shown to circulate after reinfusion into donor dogs. If alkylating agents are used to treat patients' cells, inhibition of sickling may outweigh the shortening of red cell life span induced by the treatment; blood should probably be irradiated before infusion to avoid administration of alkylated and potentially mutated, but viable, lymphocytes.


1962 ◽  
Vol 203 (3) ◽  
pp. 401-403 ◽  
Author(s):  
M. J. Cline ◽  
T. A. Waldmann

The effect of environmental temperature on erythropoiesis and erythrocyte life span was studied in frogs maintained at 4 C or 24–26 C. The red cell incorporation of radioiron was used as an index of the rate of erythropoiesis and diisopropylfluorophosphate-P32 was used to measure red cell survival. The red cell life span in frogs kept at 24–26 C was approximately 200 days. The incorporation of Fe59 into circulating red cells during the first 10 days after isotope administration was significantly greater in frogs at 24–26 C than in frogs at 4 C. The peripheral hematocrit decreased in groups of frogs kept at 4 C during the period of observation. The inconstancy of the hematocrit and presumably of the total red cell volume did not permit any definite conclusions regarding the apparently longer erythrocyte life span at low environmental temperatures.


Blood ◽  
1969 ◽  
Vol 33 (2) ◽  
pp. 214-224 ◽  
Author(s):  
PAUL R. MCCURDY

Abstract The red cell life span was measured simultaneously using 51Cr and 32DFP in 21 patients with abnormal hemoglobin β chains and in 7 patients with normal hemoglobin. The patients included 9 with sickle cell anemia, 2 with sickle-β-thalassemia disease, 2 with sickle cell-hemoglobin C disease, 3 with homozygous hemoglobin C disease, 4 with sickle cell trait and 1 with hemoglobin C trait. The 51Cr elution rate from red cells carrying abnormal β chains was 1.2 per cent daily (range: 0.1-2.7 per cent; 4 patients had 2-component elution curves, the first of which was quite rapid (4.1-19.5 per cent daily) and could lead to significant error in the 51Cr estimate of red cell survival. The 51Cr elution rate for red cells with normal β chains was 1.3 per cent daily (range: 0.7-1.6 per cent). In the steady state, production of red cells was estimated from the 32DFP life span. Both figures varied with the disease process but erythropoiesis seldom obtained the 6-8 fold increase over normal that is considered to be the capability of normal bone marrow and hence a relative erythropoietic defect seems to be frequently present in patients with abnormal hemoglobin diseases. Two sibling pairs with sickle cell anemia were studied and were found to vary from each other as much as did the other patients with this disorder.


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

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.


Sign in / Sign up

Export Citation Format

Share Document