The Hematologic System and its Disorders
Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss in Understanding Medical Terms, 2020
Similar to mismatching, erythroblastosis fetalis is caused by an antibody reaction involving Rh factor. If an Rh positive father and Rh negative mother produce an Rh positive fetus, the Rh antigens in the fetal blood reach the maternal circulation. This causes the mother to develop Rh antibodies, which can cross the placental barrier and cause agglutination and hemolysis of the fetal blood. To offset this rapid destruction, the fetus produces red cells very quickly. Because of the speed of erythrocyte production, many early nucleated cells, including erythroblasts, are released into the circulation, giving the disease its name. Unable to match production to hemolysis, many infants with erythroblastosis fetalis are stillborn. Rare in the first Rh positive infant born to an Rh negative mother, it becomes more common with successive pregnancies. Treatment relies primarily on exchange transfusion. The condition usually can be avoided by administering anti-Rh gamma-globulin immediately after the birth to prevent the mother from becoming sensitized.
Erythroblastosis fetalis
Hung N. Winn, Frank A. Chervenak, Roberto Romero in Clinical Maternal-Fetal Medicine Online, 2021
Red blood cell (RBC) alloimmunization, or isoimmunization, is the development of maternal antibodies to fetal RBC antigens. Hemolytic disease of the newborn/fetus is a hemolytic anemia that results from the lysis of the fetal RBCs by maternal antibodies. It is often characterized by excessive erythroblasts in the fetal bone marrow and circulation (erythroblastosis fetalis). Other features include generalized edema (hydrops fetalis) and hepatosplenomegaly. Alloimmunization and hemolytic disease of the newborn/fetus most commonly occur when maternal antibodies form against paternally derived Rhesus (D) antigens of the fetus, which is termed Rhesus (Rh) alloimmunization.
Embryonic and Fetal Erythropoiesis
Stephen A. Feig, Melvin H. Freedman in Clinical Disorders and Experimental Models of Erythropoietic Failure, 2019
The blood cells formed at this stage of development are overwhelmingly erythroid.21–24 The first red blood cells (RBCs), called primitive erythroblasts, appear on day 7.5 to 8.5. Primitive erythroblasts are produced intravascularly and retain a coarse nucleus throughout their life span.22–24 A second group of cells is found beginning at day 9 of gestation in the embryonic circulation.23–25 These cells are distinguished from the primitive erythroblasts by the loss of their nucleus during differentiation and by the production of hemoglobins that are the same as those found in RBCs in the fetal liver.
Red blood cells as an organ? How deep omics characterization of the most abundant cell in the human body highlights other systemic metabolic functions beyond oxygen transport
Published in Expert Review of Proteomics, 2018
Travis Nemkov, Julie A. Reisz, Yang Xia, James C. Zimring, Angelo D’Alessandro
Similarly, metabolic byproducts of RBCs could impact responses in both immature erythroblasts or mature erythrocytes. For example, purine deamidation by purine deminases (such as RBC-specific AMP deaminase 3) occurs in response to oxidative stress [69], promoting the accumulation of IMP (and its breakdown product hypoxanthine) and negatively impacting ATP/AMP ratios, thus activating AMPK and some of the responses highlighted above. Notably, hypoxanthine is released in the plasma, where it becomes a substrate for xanthine oxidase, an enzyme that generates xanthine and urate in reactions that also produce the r potent ROS hydrogen peroxide. Therefore, pro-oxidant stimuli conferred by impaired antioxidant defense in sickle cell disease, glucose 6-phosphate dehydrogenase deficiency, drug treatment, and blood bank storage could each promote hypoxanthine release and propagation of the pro-oxidant stimulus to other (red blood) cells. In so doing, urate accumulation in plasma would also result in product-induced inhibition of xanthine oxidase, thereby limiting the amplitude of the oxidative lesion to other cells. Interestingly, serum urate levels correlate with better RBC storage parameters [111], indicating that paracrine signaling in RBCs may have functional outcomes.
A Homozygous Mutation on the HBA1 Gene Coding for Hb Charlieu (HBA1: c.320T>C) Together with β-Thalassemia Trait Results in Severe Hemolytic Anemia
Published in Hemoglobin, 2019
Thomas R. L. Klei, Sima Kheradmand Kia, Martijn Veldthuis, Javad Dehbozorgian, Mehran Karimi, Judy Geissler, Erica Sellink, Marijke Thiel-Valkhof, Patrick Burger, Floris van Alphen, Alexander B. Meijer, Robin van Bruggen, Rob van Zwieten
Blood was collected from the proband, the family members and healthy controls in EDTA-containing vacutainers after informed consent was obtained, and in accordance with the Code of Ethics of the World Medical Association (Declaration of Helsinki). Erythroblasts were cultured as described previously [2]. Briefly, erythroblasts were cultured in expansion medium supplemented with stem cell factor (100 ng/mL), erythropoietin (2 U/mL; ProSpec, East Brunswick, NJ, USA) dexamethasone (1 μM; Sigma, St. Louis, MO, USA) for 2-3 weeks. Erythroblasts were then allowed to differentiate for 3 days in differentiation medium supplemented with erythropoietin (2 U/mL; ProSpec), omniplasma (5.0%) and heparin (2 UI/mL) until the basophillic/orthochromatic erythroblast stage, after which they were used for analysis.
Comparison of transcriptome profiles of nucleated red blood cells in cord blood between preterm and full-term neonates
Published in Hematology, 2022
Yuanyuan Han, Ling Huang, Man Zhou, Xiaoyu Tan, Shangjin Gong, Zhaojun Zhang, Tingting Jin, Xiangdong Fang, Yankai Jia, S. W. Huang
We compared our transcriptome data with the transcriptome datasets mentioned above [7,8,21]. These four datasets were comparisons of the transcriptomic differences of erythroblasts cultured in vitro derived from fetal livers and adult peripheral blood or bone marrow. Although the samples were from different sources, we found some overlap between these datasets (Figure 3, Table S6). For miRNA, we found that the let-7 family members were upregulated in both miRNA datasets, and that miR-675 was downregulated (Figure 3a, b). For mRNA, the adult globin genes (HBB and HBD) and transcription factor BCL11A were upregulated, whereas embryonic globin genes (HBE1 and HBZ) were downregulated in the four mRNA datasets. In addition, LIN28B, IFG2BP1, and IGF2BP3 were downregulated in three out of the four mRNA datasets (Figure 3c, d).
Related Knowledge Centers
- Fetus
- Bone Marrow
- Cellular Differentiation
- Cell Nucleus
- Hemoglobin
- Red Blood Cell
- Progenitor Cell
- Erythropoiesis
- Infant
- Complete Blood Count