scholarly journals Beta-thalassemia minor is a beneficial determinant of red blood cell storage lesion

Haematologica ◽  
2021 ◽  
Author(s):  
Vassilis L. Tzounakas ◽  
Alkmini T. Anastasiadi ◽  
Davide Stefanoni ◽  
Francesca Cendali ◽  
Lorenzo Bertolone ◽  
...  
Keyword(s):  
Blood Cell ◽  
Red Blood Cell ◽  
Pentose Phosphate ◽  
Oxidation Products ◽  
Lower Percentage ◽  
Beta Thalassemia ◽  
Potassium Accumulation ◽  
Hexosamine Pathway ◽  
Thalassemia Minor ◽  
Haematological Profile

Blood donor genetics and lifestyle affect the quality of red blood cell (RBC) storage. Heterozygotes for beta-thalassaemia (βThal+) constitute a non-negligible proportion of blood donors in the Mediterranean and other geographical areas. The unique haematological profile of βThal+ could affect capacity of enduring storage stress, however, the storability of βThal+ RBCs is largely unknown. In this study, RBCs from 18 βThal+ donors were stored in the cold and profiled for primary (haemolysis) and secondary (phosphatidylserine exposure, potassium leakage, oxidative stress) quality measures, and metabolomics, versus sex- and age-matched controls. The βThal+ units exhibited better levels of storage haemolysis and susceptibility to lysis following osmotic, oxidative and mechanical insults. Moreover, βThal+ RBCs had a lower percentage of surface removal signaling, reactive oxygen species and oxidative defects to membrane components at late stages of storage. Lower potassium accumulation and higher urate-dependent antioxidant capacity were noted in the βThal+ supernatant. Full metabolomics analyses revealed alterations in purine and arginine pathways at baseline, along with activation of pentose phosphate pathway and glycolysis upstream to pyruvate kinase in βThal+ RBCs. Upon storage, substantial changes were observed in arginine, purine and vitamin B6 metabolism, as well as in the hexosamine pathway. A high degree of glutamate generation in βThal+ RBCs was accompanied by low levels of purine oxidation products (IMP, hypoxanthine, allantoin). The βThal mutations impact the metabolism and the susceptibility to haemolysis of stored RBCs, suggesting good post-transfusion recovery. However, haemoglobin increment and other clinical outcomes of βThal+ RBC transfusion deserve elucidation by future studies.

scholarly journals A Retrospective Assessment of Prevention and Treatment of Iron Deficiency Amongst Patients Requiring Peripartum Transfusion

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1264-1264
Author(s):  
Heather Vandermeulen ◽  
Yulia Lin ◽  
Anne McLeod ◽  
Jon Barrett ◽  
Michelle Sholzberg ◽  
...  
Keyword(s):  
Iron Deficiency ◽  
Blood Cell ◽  
Red Blood Cell ◽  
Red Blood Cell Transfusion ◽  
Beta Thalassemia ◽  
Alpha Thalassemia ◽  
Iron Deficient ◽  
Thalassemia Minor ◽  
Thalassemia Trait ◽  
In Pregnancy

Abstract Background: Iron deficiency is common and affects nearly 18% of pregnant women in the United States. This is attributable to both poor baseline stores in young women and the high iron requirements of pregnancy; a singleton pregnancy results in a net loss of 630 mg of iron. Both maternal and fetal outcomes are impacted by iron deficiency. There are higher rates of maternal postpartum depression, fetal growth restriction, prematurity and developmental delay when mothers are iron deficient in pregnancy. It is also important to avoid transfusions in women of child bearing age, due to the risks of alloimmunization and hemolytic disease of the newborn. Since iron deficiency is the most common cause of anemia in pregnant women, we sought to assess the prevalence of iron deficiency in women receiving peripartum red blood cell transfusions. Materials and Methods: This study is a retrospective quality review of all cases of peripartum transfusion at an academic centre caring for high risk pregnancies from January 2013 to July 2018. All women admitted to the Labor and Delivery ward who received a red blood cell transfusion were identified through electronic blood bank database. We also identified the next age-matched woman to deliver who was not transfused. Charts were reviewed for risk factors for iron deficiency, evidence of prior iron deficiency, iron supplementation during pregnancy and fetal outcomes such as birth weight, gestational age at delivery, NICU admission and fetal mortality. A detailed transfusion history was recorded for women who received peripartum transfusions, including peritransfusion hemoglobins and indication for transfusion. Results: To date, 120 cases of peripartum red blood cell transfusion have been reviewed. Of these, 19 patients were excluded due to chronic anemia unrelated to iron deficiency or pregnancy (e.g., chronic renal failure). Age matched controls have been identified and are pending review. Preliminary data suggests that the majority of red cell transfusions given in the peripartum period are to women experiencing antepartum (26%) and/or postpartum (63%) hemorrhage. Thirty seven percent of women who were transfused had documented anemia in pregnancy and 51% of women were iron deficient in pregnancy (ferritin <30 ng/mL). Twenty one percent of women in the transfused group were noted to have pre-existing iron deficiency before conceiving. In the transfused cohort, six patients were identified as having alpha thalassemia trait (3 cases) or beta thalassemia minor (3 cases). Discussion: We present the preliminary results of a retrospective review of cases of peripartum red cell transfusion at an academic centre. Although a significant portion of transfusions were unavoidable and attributable to hemorrhage, it may be possible to decrease the number of units these women require. Over half of women who were transfused had documented iron deficiency in pregnancy. This raises the question of how many units of red blood cells could have been saved by appropriately treating these patients' iron deficiency. It is also clear in the literature that iron deficiency is associated with multiple poor fetal and maternal outcomes; we have identified an opportunity to improve the care of these women and their babies. We plan to feed this information back to the Obstetrical caregivers at our centre and to help educate providers about the recognition and treatment of iron deficiency in pregnancy. The high rate of transfusion amongst patients with alpha thalassemia trait and beta thalassemia minor warrants further investigation, but may highlight a knowledge gap around transfusion triggers for these patients. Disclosures No relevant conflicts of interest to declare.

Proposal of a score combining red blood cell indices for early differentiation of beta‐thalassemia minor from iron deficiency anemia

Hematology ◽  
2011 ◽  
Vol 16 (2) ◽  
pp. 123-127 ◽  
Author(s):  
Alexandre Janel ◽  
Laurence Roszyk ◽  
Chantal Rapatel ◽  
Gabrielle Mareynat ◽  
Marc G Berger ◽  
...  
Keyword(s):  
Iron Deficiency ◽  
Blood Cell ◽  
Iron Deficiency Anemia ◽  
Red Blood Cell ◽  
Deficiency Anemia ◽  
Beta Thalassemia ◽  
Early Differentiation ◽  
Thalassemia Minor ◽  
Red Blood Cell Indices

Prevalence of Red Blood Cell Alloimmunization Among Beta-Thalassemia and Sickle Cell Anemia Patients: a Study from Saudi Arabia

2021 ◽  
Vol 67 (10/2021) ◽  
Author(s):  
Raed Felimban ◽  
Ahmed Alsharyufi ◽  
Jasem Aljehani ◽  
Ahmed Sahlool ◽  
Hamead Aljabri ◽  
...  
Keyword(s):  
Saudi Arabia ◽  
Blood Cell ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Blood Cell ◽  
Beta Thalassemia

scholarly journals Red blood cell alloimmunisation and autoimmunisation in transfusion dependent beta thalassemics from Southern India

2015 ◽  
Vol 05 (03) ◽  
pp. 004-008
Author(s):  
Mohammed Saleem E. K. ◽  
Soundarya Mahalingam ◽  
Shamee Shastri ◽  
Kamalakshi G. Bhat
Keyword(s):  
Blood Cell ◽  
Red Blood Cell ◽  
Beta Thalassemia ◽  
Red Cell ◽  
Transfusion Therapy ◽  
Female Ratio ◽  
Antibody Screening ◽  
Cell Antigen ◽  
Low Ionic Strength ◽  
Male To Female

AbstractThe development of red blood cell (RBC) isoimmunization with alloantibodies and autoantibodies complicate transfusion therapy in multiply transfused thalassemia patients. We conducted a study to analyse the frequency in our population. Clinical and antibody profile from 55 multiply transfused thalassemic patients who were receiving transfusions were collected and analyzed prospectively. A commercially available 3 cell antigen panel was used for the antibody screening procedure. If antibody screening with the 3-cell antigen panel was positive, an extended 11-cell antigen panel was used for antibody identification in LISS (Low Ionic Strength Solution). All patients received blood matched for only ABO and Rh (D) antigens. A total of 55 transfusion dependent â thalassemics were included in this study out of which 30 (54.55%) were males and 25(45.45%) females with a male to female ratio of 1.2: 1. Frequency of red cell alloimmunization in this study was found to be 1.8%. None of the patients developed red cell autoimmunization. The alloantibody identified in the the patient who developed alloimmunisation was was anti-K. In conclusion, the transfusion of matched blood is essential for chronically transfused beta thalassemia patients in order to avoid alloimmunization.

scholarly journals Infusion of Autologous Retrodifferentiated Stem Cells into Patients with Beta-Thalassemia

2006 ◽  
Vol 6 ◽  
pp. 1278-1297 ◽  
Author(s):  
Ilham Saleh Abuljadayel ◽  
Tasnim Ahsan ◽  
Huma Quereshi ◽  
Shakil Rizvi ◽  
Tamseela Ahmed ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Blood Cell ◽  
Red Blood Cell ◽  
White Blood Cells ◽  
Fetal Hemoglobin ◽  
Beta Thalassemia ◽  
Mean Corpuscular Hemoglobin ◽  
Beta Globin ◽  
Globin Chain ◽  
Adult Hemoglobin

Beta-thalassemia is a genetic, red blood cell disorder affecting the beta-globin chain of the adult hemoglobin gene. This results in excess accumulation of unpaired alpha-chain gene products leading to reduced red blood cell life span and the development of severe anemia. Current treatment of this disease involves regular blood transfusion and adjunct chelation therapy to lower blood transfusion–induced iron overload. Fetal hemoglobin switching agents have been proposed to treat genetic blood disorders, such as sickle cell anemia and beta-thalassemia, in an effort to compensate for the dysfunctional form of the beta-globin chain in adult hemoglobin. The rationale behind this approach is to pair the excess normal alpha-globin chain with the alternative fetal gamma-chain to promote red blood cell survival and ameliorate the anemia. Reprogramming of differentiation in intact, mature, adult white blood cells in response to inclusion of monoclonal antibody CR3/43 has been described. This form of retrograde development has been termed “retrodifferentiation”, with the ability to re-express a variety of stem cell markers in a heterogeneous population of white blood cells. This form of reprogramming, or reontogeny, to a more pluripotent stem cell state ought to recapitulate early hematopoiesis and facilitate expression of a fetal and/or adult program of hemoglobin synthesis or regeneration on infusion and subsequent redifferentiation. Herein, the outcome of infusion of autologous retrodifferentiated stem cells (RSC) into 21 patients with beta-thalassemia is described. Over 6 months, Infusion of 3-h autologous RSC subjected to hematopoietic-conducive conditions into patients with beta-thalassemia reduced mean blood transfusion requirement, increased mean fetal hemoglobin synthesis, and significantly lowered mean serum ferritin. This was always accompanied by an increase in mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), and mean corpuscular hemoglobin concentration (MCHC) in such patients. No adverse side effects in response to the infusion of autologous RSC were noted.This novel clinical procedure may profoundly modify the devastating course of many genetic disorders in an autologous setting, thus paving the way to harnessing pluripotency from differentiated cells to regenerate transiently an otherwise genetically degenerate tissue such as thalassemic blood.

scholarly journals In vitro inhibition of human red blood cell acetylcholinesterase (AChE) by temephos-oxidized products

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Francisco Alberto Verdín-Betancourt ◽  
Mario Figueroa ◽  
Ma. de Lourdes López-González ◽  
Elizabeth Gómez ◽  
Yael Yvette Bernal-Hernández ◽  
...  
Keyword(s):  
Blood Cell ◽  
Red Blood Cell ◽  
Organophosphorus Pesticide ◽  
Oxidation Products ◽  
Toxicological Studies ◽  
Biological And Environmental Samples ◽  
Metabolic Products ◽  
Chlorinated Drinking Water ◽  
Oxidized Products

Abstract Temephos (Tem) is an organophosphorus pesticide widely used to kill and prevent the growth of the main vectors for the transmission of dengue, zika, and chikungunya viruses. In chlorinated water, Tem is oxidized to its dioxon-sulfoxide (Tem-dox-SO), dioxon-sulfone (Tem-dox-SO2), and sulfoxide (Tem-SO) derivatives; however, these compounds are not commercially available to be used as standards and in toxicological studies. In the present study, we synthesized and characterized the Tem-oxidation products and the compound 4,4′-sulfinyldiphenol. These compounds were obtained by a simple reaction between Tem or 4,4′-thiodiphenol with sodium hypochlorite or potassium periodate, and were characterized by IR, NMR, and UPLC-HRESIMS. The in vitro evaluation of inhibitory potency of Tem-oxidized products on human red blood cell acetylcholinesterase (RBC AChE) showed that Tem-dox-SO2 was the most potent inhibitor of human RBC AChE, and its effect was more pronounced than that observed for ethyl-paraoxon, a potent typical inhibitor of AChE. An HPLC-DAD method for the analysis of metabolic products of Tem was developed, which may be useful for monitoring in biological and environmental samples. The ability of Tem-oxidized metabolites to inhibit human RBC AChE suggests that the addition of Tem to chlorinated drinking water could result in an increase in the risk of RBC AChE inhibition after exposure.

Hemoglobin levels and red blood cell indices in mid-gestational fetuses with beta-thalassemia/HbE, beta-thalassemia trait or Hb E trait and normal fetuses

2013 ◽  
Vol 33 (13) ◽  
pp. 1238-1241
Author(s):  
Kasemsri Srisupundit ◽  
Chanane Wanapirak ◽  
Supatra Sirichotiyakul ◽  
Fuanglada Tongprasert ◽  
Suchaya Leuwan ◽  
...  
Keyword(s):  
Blood Cell ◽  
Red Blood Cell ◽  
Beta Thalassemia ◽  
Hb E ◽  
Red Blood Cell Indices ◽  
Thalassemia Trait

scholarly journals Luspatercept (Reblozyl)

2021 ◽  
Vol 1 (8) ◽  
Author(s):  
Reimbursement Team
Keyword(s):  
Cost Effectiveness ◽  
Blood Cell ◽  
Red Blood Cell ◽  
Subcutaneous Injection ◽  
Drug Class ◽  
Clinical Effectiveness ◽  
Adult Patients ◽  
Red Blood Cell Transfusion ◽  
Beta Thalassemia ◽  
Clinician Perspectives

CADTH reimbursement reviews are comprehensive assessments of the clinical effectiveness and cost-effectiveness, as well as patient and clinician perspectives, of a drug or drug class. The assessments inform non-binding recommendations that help guide the reimbursement decisions of Canada's federal, provincial, and territorial governments, with the exception of Quebec. This review assesses luspatercept (Reblozyl), 25 mg/vial, 75 mg/vial, powder for solution for subcutaneous injection. Indication: For the treatment of adult patients with red blood cell transfusion-dependent anemia associated with beta-thalassemia.

scholarly journals Simultaneous measurement of reticulocyte and red blood cell indices in healthy subjects and patients with microcytic and macrocytic anemia

Blood ◽  
1995 ◽  
Vol 85 (3) ◽  
pp. 818-823 ◽  
Author(s):  
G d'Onofrio ◽  
R Chirillo ◽  
G Zini ◽  
G Caenaro ◽  
M Tommasi ◽  
...  
Keyword(s):  
Iron Deficiency ◽  
Blood Cell ◽  
Red Blood Cell ◽  
Marrow Transplantation ◽  
Healthy Subjects ◽  
Megaloblastic Anemia ◽  
Macrocytic Anemia ◽  
Hemoglobin Concentration ◽  
Beta Thalassemia ◽  
Hemoglobin Content

Using the new Bayer H*3 hematology analyzer (Leverkusen, Germany), we have determined red blood cell and reticulocyte indices in 64 healthy subjects, in patients with microcytosis due to iron deficiency (58 patients) and heterozygous beta-thalassemia (40 patients), and in patients with macrocytosis (28 patients). We found in all cases that reticulocytes were larger than mature red cells by 24% to 35%, with a hemoglobin concentration 16% to 25% lower and a similar hemoglobin content. The correlation between red cell and reticulocyte indices was strikingly tight (r = .928 for volume, r = .929 for hemoglobin concentration, r = .972 for hemoglobin content) in all four groups, regardless of red blood cell size. The ratio of reticulocyte to red blood cell mean corpuscolar volume (MCV ratio) was constantly above 1. Inversion of the MCV ratio was observed only in four patients. It was always abrupt and transitory and was associated with erythropoietic changes leading to the production of red blood cells of a different volume (treatment of megaloblastic anemia, functional iron deficiency, bone marrow transplantation). In two cases of marrow transplantation, reticulocyte volume fell during the aplastic phase after conditioning chemotherapy and then rapidly increased up to values higher than before; this production of macroreticulocytes was the earliest sign of engraftment.

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