Recognized by erythroblastic proliferationMyeloidBone marrowImmature erythroidErythroleukemiaDifferentiationProgenitorsMaturationCellsGuglielmoMurinePrimaryVivoClassificationDiagnosisOccurPeripheralPureHighlyCasesResearchPure erythroid leukemiaPrecursorsDIFFERENTIATIONErythroleukemiaPatients with acute2016 revisionNeoplasticCellsHematopoieticAnemiaClinicalUniqueCriteria
Recognized by erythroblastic proliferation1
- PURPOSE OF THE REVIEW: Acute erythroleukemia (AEL) is a rare form of acute myeloid leukemia recognized by erythroblastic proliferation. (bvsalud.org)
Myeloid14
- Acute erythroid leukemia (AEL) is a highly aggressive subtype of acute myeloid leukemia. (bvsalud.org)
- Ever-changing diagnostic criteria and under recognition have stifled our understanding of, and therapeutic options for, this rare erythroid-predominant myeloid neoplasm. (bvsalud.org)
- Acute erythroid leukemia (AEL) is a unique subtype of acute myeloid leukemia characterized by erythroid predominance and dysplasia. (bvsalud.org)
- It is classified into two subtypes: pure erythroid (PEL) and erythroid/myeloid (EML) phenotypes. (bvsalud.org)
- RATIONALE: Pure erythroid leukemia is a rare subcategory of acute myeloid leukemia characterized by predominant immature erythroid population. (bvsalud.org)
- Erythroleukemia is a subtype of acute myeloid leukemia ( AML ) that is distinguished by erythroblastic proliferation. (medscape.com)
- [ 3 ] The 2016 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia continued to recognize pure erythroid leukemia but eliminated the erythroid/myeloid type of acute erythroleukemia. (medscape.com)
- Cases previously classified as erythroid/myeloid subtype, based on the 2008 WHO classification, are currently categorized either as myelodysplastic syndrome with excess blasts or acute myeloid leukemia, not otherwise specified. (medscape.com)
- Erythroleukemia is a neoplastic proliferation of erythroid and myeloid precursors of bone marrow hematopoietic stem cells . (medscape.com)
- However, on the basis of a study of the genetic and transcriptional landscape of 33 patients with acute erythroleukemia, Fagnan et al propose a transcriptome-based space that helps distinguish acute erythroleukemia from other myeloid leukemias. (medscape.com)
- In vivo work showed that the outcomes of disease (erythroid, myeloid, or both) depend on the driving oncogene and the hematopoietic target cell in which it is aberrantly expressed. (medscape.com)
- MEP)) and erythroid progenitors (CD71 + ) from Jak2 V617F-transduced p53-null leukemic mice had leukemia-initiating capacity, however, myeloid differentiated populations (Mac-1 + ) could not recapitulate the disease. (elsevierpure.com)
- Most of the red pulp is composed of erythroid islands, myeloid precursor cells and megakaryocytes. (cap.org)
- This clinical trial uses a laboratory test called a high throughput sensitivity assay in planning treatment for patients with relapsed or refractory acute myeloid leukemia. (clincosm.com)
Bone marrow4
- Bone marrow aspiration and biopsy are critical in making the diagnosis of acute erythroleukemia (see the image below, and Workup ). (medscape.com)
- Here we show that retroviral overexpression of Jak2 V617F mutant into wild-type p53 murine bone marrow cells induced polycythemia vera (PV) in the recipient mice, whereas Jak2 V617F-transduced p53-null mice developed lethal leukemia after the preceding PV phase. (elsevierpure.com)
- the disease is characterized by a moderate to severe macrocytic anemia, occasional neutropenia or thrombocytosis, a normocellular bone marrow with erythroid hypoplasia, and an increased risk of developing leukemia. (icdlist.com)
- a familial disorder characterized by anemia with multinuclear erythroblasts, karyorrhexis, asynchrony of nuclear and cytoplasmic maturation, and various nuclear abnormalities of bone marrow erythrocyte precursors (erythroid precursor cells). (icdlist.com)
Immature erythroid2
- It is now well-documented that true AEL, which is characterized primarily by immature erythroid proliferation, often harbors highly complex cytogenetic changes and multiple, deleterious TP53 mutations. (bvsalud.org)
- and the very uncommon pure erythroid subtype, characterized by expansion of immature erythroid cells only. (medscape.com)
Erythroleukemia8
- Dimethyl sulfoxide (DMSO) induction of mouse erythroleukemia (MEL) cells represents a well-defined in vitro system of terminal erythroid differentiation. (tmu.edu.tw)
- Reddy, PMS & Shen, CKJ 1993, ' Erythroid Differentiation of Mouse Erythroleukemia Cells Results in fr Reorganization of Protein-DNA Complexes in the Mouse β maj Globin Promoter but Not Its Distal Enhancer ', Molecular and Cellular Biology , 卷 13, 編號 2, 頁 1093-1103. (tmu.edu.tw)
- Abcb10, a mitochondrial inner membrane ATP-binding cassette transporter highly induced during erythroid maturation in hematopoietic tissues, was found as one key protein that physically interacts with Mfrn1 during mouse erythroleukemia (MEL) cell differentiation. (anthropogeny.org)
- The treatment of acute erythroleukemia is similar to that used for other subtypes of AML (see Treatment and Medication ). (medscape.com)
- Giovanni Di Guglielmo first described erythroleukemia in the early twentieth century, and the disorder is often still referred to as acute Di Guglielmo syndrome. (medscape.com)
- Erythroleukemia shares clinical and pathologic features with myelodysplastic syndromes , especially with erythroid-predominant myelodysplastic syndromes. (medscape.com)
- Further work by Fagnan et al in an animal model found that aberrant expression of oncogenic driver transcription factors results in erythroleukemia by downregulating the GATA1-regulated erythroid epigenome. (medscape.com)
- The latter phase of disease is characterized by a clonal expansion of transformed leukemic cells causing an acute erythroleukemia in mice. (psu.edu)
Differentiation2
- Our genomic footprinting data have interesting implications with respect to the molecular mechanisms of transcriptional regulation and chromatin change of the mouse β maj globin gene during erythroid differentiation. (tmu.edu.tw)
- The macrophage contained within the erythroblastic island is central to the normal differentiation and support of erythroid development. (edu.au)
Progenitors3
- Sf-Stk provides signals that cooperate with EpoR signals to effect expansion of erythroid progenitors. (psu.edu)
- Signaling by Sf-Stk and EpoR mediated by gp55 renders erythroid progenitors Epo independent through the activation of the EpoR downstream pathways such as PI3K, MAPK and JAK/STAT. Previous work has shown that Src family kinases also play an important role in erythropoiesis. (psu.edu)
- The leukemic mice had severe anemia, hepatosplenomegaly, pulmonary hemorrhage and expansion of dysplastic erythroid progenitors. (elsevierpure.com)
Maturation1
- The erythroid islands show normoblastic maturation. (cap.org)
Cells3
- Interestingly, recipients transplanted with CD71 + cells rapidly developed erythroid leukemia, which was in sharp contrast to leukemic KSL cells to cause lethal leukemia after the polycythemic state. (elsevierpure.com)
- Our results provide strong evidence that the theta 1-globin gene of humans is transcriptionally active in cells of erythroid origin, and suggests the presence of a functional theta 1-polypeptide in specific cells, possibly those of early erythroid tissue. (ox.ac.uk)
- The aim is to try to identify drugs that may be effective in killing leukemia cells for those patients who will not be cured with conventional chemotherapy. (clincosm.com)
Guglielmo1
- Pure erythroid malignancies, such as Di Guglielmo disease (DG). (elsevierpure.com)
Murine1
- METHODS: We evaluated leukocyte recruitment in vivo by using real-time multichannel fluorescence intravital microscopy of a tumor necrosis factor-α-induced acute inflammation model in both murine arterial and venous macrovasculature and microvasculature. (bioxcell.com)
Primary2
- Primary acute erythroid leukemia is associated with complex and high-risk karyotypes including chromosomes 5q and 7q abnormalities. (bvsalud.org)
- Splenomegaly can be seen in many cases of myeloproliferative neoplasm-including chronic myelogeneous leukemia, primary myelofibrosis, polycythemia vera and essential thrombocytosis. (cap.org)
Vivo1
- RESULTS: In vivo quantitative imaging analyses of acute inflammation revealed a 24-hour rhythm in leukocyte recruitment to arteries and veins of the mouse macrovasculature and microvasculature. (bioxcell.com)
Classification1
- in which the predominant immature elements are proerythroblasts, are excluded from the French-American-British (FAB) classification for acute leukemia and do not fit neatly into any of the categories of myelodysplasia. (elsevierpure.com)
Diagnosis1
- Many controversies remain around diagnosis influencing prognostic and therapeutic implications relating to this unique leukemia subset. (bvsalud.org)
Occur1
- Progressive chronic renal failure can occur after several years of hemoglobinuria from the acute tubulonecrosis effects of heme and iron (pigment nephropathy), decreased renal perfusion from renal vein thrombosis, and tubular obstruction with pigment casts. (medscape.com)
Peripheral1
- His peripheral blood smear shows a leuko-erythroblastic picture. (cap.org)
Pure1
- Since the first recognition of an erythroid-predominant hematologic malignancy in the early 20th century, AEL has gone through a turnstile of changing definitions and nomenclature, including eritoleucemia, erythremic myelosis, AML-M6 and pure erythroid leukemia. (bvsalud.org)
Highly1
- BACKGROUND: The incidence of acute cardiovascular complications is highly time-of-day dependent. (bioxcell.com)
Cases1
- To understand the mechanism of the erythroid dominant phenotype of AEL and identify potential therapeutic targets for AEL, we analyzed 105 AEL and 214 non-AEL cases using whole-genome/exome and/or targeted-capture sequencing, with SNP probes for detecting copy number abnormalities. (bvsalud.org)
Research1
- Leukemia Research , 30 (9), 1141-1149. (psu.edu)
Pure erythroid leukemia6
- Pure erythroid leukemia (PEL) is a rare acute leukemia with a dismal prognosis. (nih.gov)
- 3. Pure erythroid leukemia: a reassessment of the entity using the 2008 World Health Organization classification. (nih.gov)
- 6. Pure Erythroid Leukemia and Erythroblastic Sarcoma Evolving From Chronic Myeloid Neoplasms. (nih.gov)
- 9. The uniqueness of morphological features of pure erythroid leukemia in myeloid neoplasm with erythroid predominance: A reassessment using criteria revised in the 2016 World Health Organization classification. (nih.gov)
- 16. De novo pure erythroid leukemia: refining the clinicopathologic and cytogenetic characteristics of a rare entity. (nih.gov)
- 3] The 2016 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia continued to recognize pure erythroid leukemia but eliminated the erythroid/myeloid type of acute erythroleukemia. (medscape.com)
Precursors2
- Erythroid precursors differentiate sequentially from stem cells to progenitor cells to erythroblasts to normoblasts in a process requiring growth factors and cytokines. (medscape.com)
- Normally, erythroid precursors are released into circulation as reticulocytes. (medscape.com)
DIFFERENTIATION1
- Geller, R , Levenson, R & Housman, D 1978, ' Significance of the cell cycle in commitment of murine erythroleukemia cells to erythroid differentiation ', Journal of Cellular Physiology , vol. 95, no. 2, pp. 213-222. (psu.edu)
Erythroleukemia12
- Vitamin B12 and folate - Severe pernicious anemia sometimes mimics acute erythroleukemia. (medscape.com)
- Erythroleukemia is required to have both erythroblastic and myeloblastic components (see the image below). (medscape.com)
- Erythroid markers such as glycophorin A and transferrin receptor (CD71 and CD45) may be increased, but they are negative in many patients with erythroleukemia. (medscape.com)
- Therefore, whereas the expression of glycophorin A and/or transferrin receptor may be helpful, the absence of erythroid antigens does not exclude erythroleukemia. (medscape.com)
- Erythroleukemia is a subtype of acute myeloid leukemia (AML) that is distinguished by erythroblastic proliferation. (medscape.com)
- The treatment of acute erythroleukemia is similar to that used for other subtypes of AML (see Treatment and Medication). (medscape.com)
- Giovanni Di Guglielmo first described erythroleukemia in the early twentieth century, and the disorder is often still referred to as acute Di Guglielmo syndrome. (medscape.com)
- Erythroleukemia shares clinical and pathologic features with myelodysplastic syndromes, especially with erythroid-predominant myelodysplastic syndromes. (medscape.com)
- However, on the basis of a study of the genetic and transcriptional landscape of 33 patients with acute erythroleukemia, Fagnan et al propose a transcriptome-based space that helps distinguish acute erythroleukemia from other myeloid leukemias. (medscape.com)
- Further work by Fagnan et al in an animal model found that aberrant expression of oncogenic driver transcription factors results in erythroleukemia by downregulating the GATA1-regulated erythroid epigenome. (medscape.com)
- Acute erythroleukemia accounts for 3-5% of all de novo AMLs and 20-30% of secondary leukemias. (medscape.com)
- 2, 10] Although very rare in children, acute erythroleukemia has been reported in children from the newborn period through age 7 years. (medscape.com)
Patients with acute1
- 17. Study of clinical, haematological and cytogenetic profile of patients with acute erythroid leukaemia. (nih.gov)
2016 revision1
- 8. An analysis of 97 previously diagnosed de novo adult acute erythroid leukemia patients following the 2016 revision to World Health Organization classification. (nih.gov)
Neoplastic1
- 2. Acute erythroid neoplastic proliferations. (nih.gov)
Cells2
- FAB criteria require (1) a 50% or more erythroid component in all nucleated cells and (2) at least one of the following: 30% or more nonerythroid blasts, excluding erythroblasts, or less than 30% blasts in all nucleated cells. (medscape.com)
- [ 19 ] the leukemic cells often express both erythroid and myeloid markers. (medscape.com)
Hematopoietic2
- In vivo work showed that the outcomes of disease (erythroid, myeloid, or both) depend on the driving oncogene and the hematopoietic target cell in which it is aberrantly expressed. (medscape.com)
- Allogeneic hematopoietic stem cell transplantation is curative but it requires a histocompatible donor and is associated with significant morbidity and mortality, so it is reserved for severe cases of PNH with aplastic anemia or transformation to leukemia. (medscape.com)
Anemia1
- With anemia due to acute blood loss, a reduction in oxygen-carrying capacity occurs along with a decrease in intravascular volume, with resultant hypoxia and hypovolemia. (medscape.com)
Clinical1
- 1. Acute leukaemia with a pure erythroid phenotype: under-recognized morphological and cytogenetic signatures associated universally with primary refractory disease and a dismal clinical outcome. (nih.gov)
Unique1
- 5. How unique is pure erythroid leukaemia? (nih.gov)