scholarly journals Erythropoietin-dependent primary pure erythrocytosis

Blood ◽  
1979 ◽  
Vol 53 (6) ◽  
pp. 1076-1084 ◽  
Author(s):  
N Dainiak ◽  
R Hoffman ◽  
AI Lebowitz ◽  
L Solomon ◽  
L Maffei ◽  
...  

Abstract We investigated the pathogenesis of isolated erythrocytosis of 14 yr duration in a 28-yr-old man. The increase in red cell mass was attributed to increased erythropoietin production. An extensive search for recognized causes of secondary erythrocytosis was unrevealing. Family members were found to be hematologically normal. After reduction of the circulating red cell mass by 20%, erythropoietin activity nearly quadrupled, thus suggesting a normal erythropoietin response to phlebotomy. When bone marrow cells of the patient were cultured in plasma clots in the absence of added erythropoietin, endogenous erythroid colony formation was observed, a pattern previously believed to be specific for polycythemia vera bone marrow cells. Our observations suggest that the erythrocytosis in this individual is best explained by an abnormal “servoregulatory” mechanism of erythropoietin production. In addition, this is the first instance in which the rule that endogenous erythroid colony formation is correlated with the diagnosis of polycythemia vera has not held.

Blood ◽  
1979 ◽  
Vol 53 (6) ◽  
pp. 1076-1084 ◽  
Author(s):  
N Dainiak ◽  
R Hoffman ◽  
AI Lebowitz ◽  
L Solomon ◽  
L Maffei ◽  
...  

We investigated the pathogenesis of isolated erythrocytosis of 14 yr duration in a 28-yr-old man. The increase in red cell mass was attributed to increased erythropoietin production. An extensive search for recognized causes of secondary erythrocytosis was unrevealing. Family members were found to be hematologically normal. After reduction of the circulating red cell mass by 20%, erythropoietin activity nearly quadrupled, thus suggesting a normal erythropoietin response to phlebotomy. When bone marrow cells of the patient were cultured in plasma clots in the absence of added erythropoietin, endogenous erythroid colony formation was observed, a pattern previously believed to be specific for polycythemia vera bone marrow cells. Our observations suggest that the erythrocytosis in this individual is best explained by an abnormal “servoregulatory” mechanism of erythropoietin production. In addition, this is the first instance in which the rule that endogenous erythroid colony formation is correlated with the diagnosis of polycythemia vera has not held.


1987 ◽  
Vol 252 (2) ◽  
pp. R216-R221 ◽  
Author(s):  
R. D. Lange ◽  
R. B. Andrews ◽  
L. A. Gibson ◽  
C. C. Congdon ◽  
P. Wright ◽  
...  

Previous studies have shown that a decrease in red cell mass occurs in astronauts, and some studies indicate a leukocytosis occurs. A life science module housing young and mature rats was flown on shuttle mission Spacelab 3 (SL-3), and the results of hematology studies of flight and control rats are presented. Statistically significant increases in the hematocrit, red blood cell counts, and hemoglobin determinations, together with a mild neutrophilia and lymphopenia, were found in flight animals. No significant changes were found in bone marrow and spleen cell differentials or erythropoietin determinations. Clonal assays demonstrated an increased erythroid colony formation of flight animal bone marrow cells at erythropoietin doses of 0.02 and 1.0 U/ml but not 0.20 U/ml. These results agree with some but vary from other previously published studies. Erythropoietin assays and clonal studies were performed for the first time.


2021 ◽  
Vol 2 (2) ◽  
pp. 75-81
Author(s):  
Milica Jeremić ◽  
Danijela Leković ◽  
Dijana Šefer ◽  
Vesna Đorđević ◽  
Andrija Bogdanović

Introduction: Erythrocytosis represents elevated hemoglobin and hematocrit levels above the range of normal values. Primary erythrocytosis - polycythemia vera, is characterized by increased erythrocyte production, due to a disorder at the level of the multipotent stem cell in the bone marrow. On the other hand, secondary erythrocytosis (SE) is the result of bone marrow stimulation by an external factor. Aim: The aim of our study was to determine parameters which are significant in differentiating SE from primary erythrocytosis - polycythemia vera (PV). Materials and methods: This is a retrospective study involving 108 patients with SE and 111 patients with PV, who were diagnosed and treated at the Clinic of Hematology of the Clinical Center of Serbia (CCS), in the period: December 2005 - November 2018.From the patient records, the following data were extracted: demographic characteristics, laboratory parameters, spleen size, total red cell mass, serum erythropoietin (EPO) level, and spontaneous growth of the BFU-E colony. Results: Patients with SE were younger, with a predominance of the male gender and with significantly higher serum EPO values than patients with PV. Patients with PV had significantly higher values of BFU-E, leukocyte and platelet count, spleen size, and LDH level than patients with SE. Total red cell mass analysis did not show a differential diagnostic significance. Conclusion: Findings of normal spleen size, normal leukocyte and platelet count, normal serum LDH level, and elevated EPO, in patients, refer to the diagnosis of secondary erythrocytosis, while the findings of splenomegaly, leukocytosis, thrombocytosis, elevated serum LDH level, decreased EPO, and the presence of spontaneous BFU-E colony speak in favor of the diagnosis of polycythemia vera.


1985 ◽  
Vol 60 (1) ◽  
pp. 129-136 ◽  
Author(s):  
M. Y. Gordon ◽  
J. A. Hibbin ◽  
L. U. Kearney ◽  
E. C. Gordon-Smith ◽  
J. M. Goldman

Blood ◽  
1984 ◽  
Vol 63 (4) ◽  
pp. 784-788 ◽  
Author(s):  
VF LaRussa ◽  
F Sieber ◽  
LL Sensenbrenner ◽  
SJ Sharkis

Abstract In this article, we present evidence that sialic acid-containing surface components play a role in the regulation of erythropoiesis. A 1- hr exposure of mouse bone marrow cells to high concentrations of neuraminidase reduced erythroid colony formation. Coculture of 10(6) untreated thymocytes with neuraminidase-treated bone marrow cells restored erythroid colony growth. Neuraminidase-treated thymocytes retained their ability to suppress erythroid colony formation by untreated marrow cells, but lost their ability to enhance erythroid colony formation. Continuous exposure to low concentrations of neuraminidase enhanced erythroid bone marrow cell colony growth in response to a suboptimal dose of erythropoietin.


1989 ◽  
Vol 256 (4) ◽  
pp. C925-C929 ◽  
Author(s):  
I. Seferynska ◽  
J. Brookins ◽  
J. C. Rice ◽  
J. W. Fisher

Our present study was undertaken to determine the serum erythropoietin concentration (radioimmunoassay), hematocrit, red cell mass, and body weight of mice exposed to hypoxia in a hypobaric chamber (0.42 atm, 22 h/day) for 14 days and during the 10 posthypoxic days at ambient pressure to clarify the correlation of the red cell mass and erythropoietin production during hypoxia. The mean serum erythropoietin titer was 326.23 +/- 77.04 mU/ml after 2 days, reached the highest level after 3 days (452.2 +/- 114.5 mU/ml), then gradually declined to a level of 36.5 +/- 11.4 mU/ml after 14 days of hypoxia, and was undetectable during the 10-day posthypoxic period. The hematocrit values were significantly increased from 41.09 +/- 0.50% at day 0 to 51.65 +/- 1.08% after 3 days and to 72.20 +/- 1.53% after 14 days of hypoxia. The red cell mass (calculated from initial body weight) increased from 3.24 +/- 0.1 ml/100 g at day 0 to 7.32 +/- 0.46 ml/100 g after 14 days of hypoxia and declined to 6.66 +/- 0.53 ml/100 g at the end of the 10-day posthypoxic period. The mice lost weight while they were in the hypobaric chamber and showed a significant increase in body weight during the 10-day posthypoxic period. These studies support the concept that chronic intermittent hypoxia causes an early increase, followed by a rapid decline, in erythropoietin production, which is correlated with the gradual increase in red cell mass.


Blood ◽  
1964 ◽  
Vol 24 (3) ◽  
pp. 267-280 ◽  
Author(s):  
S.-AÅ. KILLMANN ◽  
E. P. CRONKITE ◽  
T. M. FLIEDNER ◽  
V. P. BOND

Abstract 1. Weighted average mitotic time of human bone marrow cells is estimated. Mitotic time is about 0.75 hours in red cell precursors and about 0.58 hours in neutrophil precursors. 2. Estimated weighted average DNA-synthesis time is about 9 hours in red cell precursors and about 24 hours in neutrophil precursors. 3. Upper and lower limits for compartment transit times of proerythroblasts, basophilic normoblasts, polychromatic normoblasts, myeloblasts, promyelocytes and myelocytes are presented (table 2). 4. Total mass of nucleated red cell precursors is estimated at 3.5 x 109/kg. and total mass of neutrophils and their precursors in the marrow at 8.8 x 109/kg.


Blood ◽  
1972 ◽  
Vol 40 (3) ◽  
pp. 353-365 ◽  
Author(s):  
Raymond Alexanian ◽  
Judith Nadell ◽  
Clarence Alfrey

Abstract Oxymetholone was given to 28 adults with chronic anemia from bone marrow disease. Changes in hematocrit and red cell mass were correlated with serial assessments of erythropoietin and erythropoiesis. Erythropoietin excretion was enhanced more than five-fold over the level expected for the hematocrit in 70% of the patients. Only 23% of the patients with an evaluable treatment trial increased their red cell mass by at least 20%. In all responders, the T½ of 59Fe disappearance ranged from 86-136 min and erythron iron turnover exceeded 0.25 mg/100 ml blood/day. A decline in serum iron concentration to the 50-100 µg/100 ml range after 1 mo of oxymetholone was frequently associated with a subsequent response to therapy. Patients with severe bone marrow failure, for whom frequent red cell transfusions were required, did not improve. The failure of other patients to respond was attributed to complicating factors that either impaired maximal erythropoietin production or restricted iron supply to the bone marrow. Hepatic toxicity was detected in less than 10% of treated patients. Results support the use of oxymetholone in the treatment of patients with moderate degrees of bone marrow failure and symptomatic anemia.


Blood ◽  
1951 ◽  
Vol 6 (11) ◽  
pp. 1021-1033 ◽  
Author(s):  
FREDERICK ROSS BIRKHILL ◽  
MARY A. MALONEY ◽  
STANLEY M. LEVENSON

Abstract 1. The effect of transfusion polycythemia upon bone marrow activity and erythrocyte survival has been studied in 4 normal young adult males. 2. Plasma volumes did not change significantly throughout the period of study. 3. Total red cell masses increased to the "expected" levels, i.e., to the total of the subjects’ cells plus the transfused cells immediately after the transfusions. Thereafter these fell progressively, reaching the control levels in about forty days. 4. Survival of the infused cells was normal. 5. The subjects’ own red cell masses fell progressively at first. This was due to decreased erythropoiesis rather than to increased destruction. This is indicated by (a) no consistent significant elevation in hemolytic indexes; (b) change of the myeloid-erythroid ratio from normal values of 4 to 7:1 to about 20:1 two weeks post-infusion; (c) consistent decrease in circulating reticulocytes. 6. The depression of erythropoiesis was directly related to the quantity of red cells infused; almost complete cessation of red cell synthesis followed an increase of the red cell mass by forty per cent. 7. The depression of erythropoiesis was only temporary. As soon as the total circulating red cell mass returned to the pre-injection level, erythropoiesis proceeded at a normal rate. 8. No consistent changes in the circulating white blood cells, totals and differentials were noted. 9. Mild abnormalities in some liver functions were observed. Whether these should be attributed to the effects of the transfusions directly, or to the mild febrile responses experienced by the subjects shortly after the infusions cannot be stated with certainty at present.


Sign in / Sign up

Export Citation Format

Share Document