scholarly journals Deformability of transfused red blood cells is a potent effector of transfusion-induced hemoglobin increment: A study with β-thalassemia major patients

2017 ◽  
Vol 92 (9) ◽  
pp. E559-E560 ◽  
Author(s):  
Gregory Barshtein ◽  
Neta Goldschmidt ◽  
Axel R Pries ◽  
Orly Zelig ◽  
Dan Arbell ◽  
...  
Keyword(s):  
Red Blood Cells ◽  
Blood Cells ◽  
Thalassemia Major ◽  
Potent Effector

scholarly journals Hematopoietic Peripheral Circulating Blood Stem Cells As an Independent Marker of Good Transfusion Management in Patients with Beta-Thalassemia

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 957-957
Author(s):  
Mariasanta Napolitano ◽  
Calogera Gerardi ◽  
Anna Di Lucia ◽  
Pietro Andrea Accardo ◽  
Luigi Rizzuto ◽  
...  
Keyword(s):  
Stem Cells ◽  
Stem Cell ◽  
Red Blood Cells ◽  
Blood Cells ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Healthy Controls ◽  
Thalassemia Intermedia ◽  
Cd34 Cells ◽  
Independent Marker

Abstract Aim Aim of the current study was to prospectively evaluate the potential role of peripheral circulating CD34+ stem cells as new independent marker of appropriate hemopoietic balance in patients with thalassemia major and intermedia. Materials and methods Peripheral blood samples from patients with thalassemia major (TM) and intermedia (TI) were drawn. Peripheral circulating CD34+ stem cells, CF-GEMM, CFU-GM and BFU-GM were assayed with monoclonal antibodies for CD34 and clonogenic tests, according to standard procedures and ISHAGE method (BD stem cell enumeration kit, Becton Dickinson; H4434, Stem Cell Technology). Demographic and clinical data were recorded from each enrolled subject. Results were compared with healthy controls. Results Overall, 56 patients with thalassemia major (median age:35 years, range:13-52 years) and 13 with thalassemia intermedia (median age:44 years, range:27-67) were evaluated. Annual red blood cells transfusion requirements ranged from 10 to 65 in all the patients except three with thalassemia intermedia, that were transfusion independent. One patient with TM did not accept transfusion for religion (Table 1). A statistically significant increase in peripheral circulating stem cells was observed in all the patients, in comparison with healthy controls. Mean peripheral circulating CD34+cells were 6.9±4.5/mmc in patients with TM and 11.8±14.8/mmc in patients with TI, significantly higher than mean CD34+ cells of healthy volunteer blood donors values (3.5±2.9/mmc, p=0.014 and p= 0.051 respectively for TM and TI). Only in patients with TI an increase in CFU-GEMM (3.0±4.8 vs 0.75±2.05, p=0.0001) was observed. Patients not treated with transfusions showed the mean highest levels of circulating stem cells (CD34: 32.5±14.8/mmc, BFU-E: 41.3±22.8, CFU-GM: 19.6±5.6, CFU-GEMM 9.0±6.1). At multivariate analysis, peripheral circulating CD34+ stem cells did not correlate with age, sex, number of red blood cells units transfused, hemoglobin levels, history of splenectomy and hypothyroidism. Conclusion Circulating peripheral CD34+stem cells are increased in patients with the highest hemopoietic stress (TI, transfusion independent), thus their determination could represent a useful independent marker of clinical response to transfusion in patients with beta thalassemia. Table 1. Baseline data of patients with thalassemia Thalassemia major (n=56) Thalassemia intermedia (n=13) Males 28 7 Females 28 6 Mean Age (range) 35 (13-52) 42(27-67) BMI (range) 21.5 (15.7-27.3) 21.4 (14.7-33.5) Splenectomy 31 13 Hypothyroidism 9 1 Smokers 32 31 Disclosures No relevant conflicts of interest to declare.

scholarly journals Effects of α-tocopherol on hemolysis and oxidative stress markers on red blood cells in β-thalassemia major

2020 ◽  
Vol 63 (8) ◽  
pp. 314-320
Author(s):  
Nora Sovira ◽  
Munar Lubis ◽  
Pustika Amalia Wahidiyat ◽  
Franciscus D. Suyatna ◽  
Djajadiman Gatot ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Red Blood Cells ◽  
Blood Cells ◽  
Controlled Trial ◽  
Osmotic Fragility ◽  
Thalassemia Major ◽  
Oxidative Stress Markers ◽  
Institute Of Medicine ◽  
Stress Markers ◽  
And Oxidative Stress

Background: The accumulation of unpaired α-globin chains in patients with β-thalassemia major may clinically create ineffective erythropoiesis, hemolysis, and chronic anemia. Multiple blood transfusions and iron overload cause cellular oxidative damage. However, α-tocopherol, an antioxidant, is a potent scavenger of lipid radicals in the membranes of red blood cells (RBCs) of patients with β-thalassemia major.Purpose: To evaluate the effects of α-tocopherol on hemolysis and oxidative stress markers on the RBC membranes of patients with β-thalassemia major.Methods: Forty subjects included in this randomized controlled trial were allocated to the placebo and α-tocopherol groups. Doses of α-tocopherol were based on Institute of Medicine recommendations: 4–8 years old, 200 mg/day; 9–13 years old, 400 mg/day; 14–18 years old, 600 mg/day. Hemolysis, oxidative stress, and antioxidant variables were evaluated before and after 4-week α-tocopherol or placebo treatment, performed before blood transfusions.Results: Significant enhancements in plasma haptoglobin were noted in the α-tocopherol group (3.01 mg/dL; range, 0.60–42.42 mg/dL; <i>P</i>=0.021). However, there was no significant intergroup difference in osmotic fragility test results; hemopexin, malondialdehyde, reduced glutathione (GSH), or oxidized glutathione (GSSG) levels; or GSH/GSSG ratio.Conclusion: Use of α-tocopherol could indirectly improve hemolysis and haptoglobin levels. However, it played no significant role in oxidative stress or as an endogen antioxidant marker in β-thalassemia major.

S17.1. Enhanced aggregability of red blood cells of β-thalassemia major patients; Monitoring by computerized image analysis

Biorheology ◽  
1995 ◽  
Vol 32 (2-3) ◽  
pp. 166-166
Author(s):  
S CHEN ◽  
A ELDOR ◽  
G BARSHTEIN ◽  
S ZHANG ◽  
A GOLDFARB ◽  
...  
Keyword(s):  
Image Analysis ◽  
Red Blood Cells ◽  
Blood Cells ◽  
Thalassemia Major ◽  
Computerized Image Analysis

scholarly journals PS1574 HLA-DRB1∗15 INCREASES THE RISK OF RED BLOOD CELLS ALLOANTIBODIES IN PATIENTS WITH BETA THALASSEMIA MAJOR

HemaSphere ◽  
2019 ◽  
Vol 3 (S1) ◽  
pp. 725-726
Author(s):  
L.A. Al Mashaykhi ◽  
M.K. Al-Khabori ◽  
H. Al-Riyami ◽  
B. Al-Rawahi ◽  
Y. Wali ◽  
...  
Keyword(s):  
Red Blood Cells ◽  
Blood Cells ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major

Compensation of CD55 Underexpression on Red Blood Cells ofβ-Thalassemia Major Patients

Hemoglobin ◽  
2015 ◽  
Vol 39 (3) ◽  
pp. 184-189 ◽  
Author(s):  
Jamil M. A. S. Obaid ◽  
Salma Y. Abo El-Nazar ◽  
Amal M. Ghanem ◽  
Abeer S. El-Hadidi ◽  
Basma H. M. Mersal
Keyword(s):  
Red Blood Cells ◽  
Blood Cells ◽  
Thalassemia Major

scholarly journals Studies on the Fetal Hemoglobin in the Persistent High Hb-F Anomaly

Blood ◽  
1961 ◽  
Vol 18 (3) ◽  
pp. 267-284 ◽  
Author(s):  
R. B. THOMPSON ◽  
J. W. MITCHENER ◽  
TITUS H. J. HUISMAN
Keyword(s):  
Red Blood Cells ◽  
Blood Cells ◽  
Fetal Hemoglobin ◽  
Thalassemia Major ◽  
Structural Investigations ◽  
Distinct Cell ◽  
Abnormal Hemoglobin ◽  
Newborn Child ◽  
Hb S ◽  
The Individual

Abstract Three generations of a Negro family having herditary persistence of fetal hemoglobin in conjunction with the hemoglobins A and S were studied. Genetic studies did not exclude the possibility that this anomaly is allelic with the genes responsible for the hemoglobins A, S, and C. Structural investigations of the isolated abnormal hemoglobin fraction offered evidence that this component is identical with the fetal hemoglobin of the newborn child. No striking clinical or hematologic abnormalities were found in members of the family who had high percentages of fetal hemoglobin in conjunction with either Hb-A or Hb-S. The per cent of Hb-A2 in the persistent high Hb-F carriers was found slightly below normal, while the total amount of circulating hemoglobins was increased. Previous reports of the complete absence of Hb-A in cases heterozygous for Hb-S as well as for the persistent high Hb-F anomaly have been confirmed by using refined technics available for hemoglobin analyses. The fetal hemoglobin was distributed almost equally in all erythrocytes of individuals having this inherited anomaly. In this respect, the S-F abnormality differs from homozygous sickle-cell disease in which a mixed population of red blood cells, some with and most without Hb-F, has been demonstrated. It is suggested that the presence of some Hb-F in all erythrocytes of the individual with the S-F condition is the factor that protects the cells from sickling and from destruction. The distribution of fetal hemoglobin in the red blood cells of persistent high Hb-F carriers was also different from that found in patients with thalassemia major and thalassemia-Hb-E disease. Two distinct cell populations, one with and the other without fetal hemoglobin, were found to be present in the blood of the thalessemia patients.

scholarly journals EVALUATION OF IRON OVERLOAD AND ADEQUACY OF PACKED RED BLOOD CELLS TRANSFUSION IN CHILDREN WITH THALASSEMIA MAJOR

2019 ◽  
Vol 06 (09) ◽  
pp. 495-498
Author(s):  
Santosh Kumar Shukla ◽  
Manisha Maurya ◽  
Anubha Shrivastava ◽  
Prakash Chandra Mishra
Keyword(s):  
Iron Overload ◽  
Red Blood Cells ◽  
Blood Cells ◽  
Thalassemia Major ◽  
Packed Red Blood Cells
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