Aplastic Anemia as a Roadmap for Bone Marrow Failure: An Overview and a Clinical Workflow
In recent years, it has become increasingly apparent that bone marrow (BM) failures and myeloid malignancy predisposition syndromes are characterized by a wide phenotypic spectrum and that these diseases must be considered in the differential diagnosis of children and adults with unexplained hematop...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-10-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | https://www.mdpi.com/1422-0067/23/19/11765 |
| _version_ | 1797478894959329280 |
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| author | Antonio G. Solimando Carmen Palumbo Mary Victoria Pragnell Max Bittrich Antonella Argentiero Markus Krebs |
| author_facet | Antonio G. Solimando Carmen Palumbo Mary Victoria Pragnell Max Bittrich Antonella Argentiero Markus Krebs |
| author_sort | Antonio G. Solimando |
| collection | DOAJ |
| description | In recent years, it has become increasingly apparent that bone marrow (BM) failures and myeloid malignancy predisposition syndromes are characterized by a wide phenotypic spectrum and that these diseases must be considered in the differential diagnosis of children and adults with unexplained hematopoiesis defects. Clinically, hypocellular BM failure still represents a challenge in pathobiology-guided treatment. There are three fundamental topics that emerged from our review of the existing data. An exogenous stressor, an immune defect, and a constitutional genetic defect fuel a vicious cycle of hematopoietic stem cells, immune niches, and stroma compartments. A wide phenotypic spectrum exists for inherited and acquired BM failures and predispositions to myeloid malignancies. In order to effectively manage patients, it is crucial to establish the right diagnosis. New theragnostic windows can be revealed by exploring BM failure pathomechanisms. |
| first_indexed | 2024-03-09T21:38:06Z |
| format | Article |
| id | doaj.art-4516e897c8bf498eafca7cfee7ac6530 |
| institution | Directory Open Access Journal |
| issn | 1661-6596 1422-0067 |
| language | English |
| last_indexed | 2024-03-09T21:38:06Z |
| publishDate | 2022-10-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | International Journal of Molecular Sciences |
| spelling | doaj.art-4516e897c8bf498eafca7cfee7ac65302023-11-23T20:38:50ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672022-10-0123191176510.3390/ijms231911765Aplastic Anemia as a Roadmap for Bone Marrow Failure: An Overview and a Clinical WorkflowAntonio G. Solimando0Carmen Palumbo1Mary Victoria Pragnell2Max Bittrich3Antonella Argentiero4Markus Krebs5Department of Biomedical Sciences and Human Oncology, Section of Internal Medicine ‘G. Baccelli’, University of Bari Aldo Moro Medical School, 70124 Bari, ItalyDepartment of Biomedical Sciences and Human Oncology, Section of Internal Medicine ‘G. Baccelli’, University of Bari Aldo Moro Medical School, 70124 Bari, ItalySection of Pathology, Department of Emergency and Organ Transplantation (DETO), University of Bari Aldo Moro, 70124 Bari, ItalyDepartment of Internal Medicine II, University Hospital Würzburg, 97080 Würzburg, GermanyIRCCS Istituto Tumori “Giovanni Paolo II” of Bari, 70124 Bari, ItalyComprehensive Cancer Center Mainfranken, University Hospital Würzburg, 97080 Würzburg, GermanyIn recent years, it has become increasingly apparent that bone marrow (BM) failures and myeloid malignancy predisposition syndromes are characterized by a wide phenotypic spectrum and that these diseases must be considered in the differential diagnosis of children and adults with unexplained hematopoiesis defects. Clinically, hypocellular BM failure still represents a challenge in pathobiology-guided treatment. There are three fundamental topics that emerged from our review of the existing data. An exogenous stressor, an immune defect, and a constitutional genetic defect fuel a vicious cycle of hematopoietic stem cells, immune niches, and stroma compartments. A wide phenotypic spectrum exists for inherited and acquired BM failures and predispositions to myeloid malignancies. In order to effectively manage patients, it is crucial to establish the right diagnosis. New theragnostic windows can be revealed by exploring BM failure pathomechanisms.https://www.mdpi.com/1422-0067/23/19/11765hematopoietic stem cellsbone marrow immune-microenvironmentbone marrow failurecytopeniaaplastic anemia |
| spellingShingle | Antonio G. Solimando Carmen Palumbo Mary Victoria Pragnell Max Bittrich Antonella Argentiero Markus Krebs Aplastic Anemia as a Roadmap for Bone Marrow Failure: An Overview and a Clinical Workflow International Journal of Molecular Sciences hematopoietic stem cells bone marrow immune-microenvironment bone marrow failure cytopenia aplastic anemia |
| title | Aplastic Anemia as a Roadmap for Bone Marrow Failure: An Overview and a Clinical Workflow |
| title_full | Aplastic Anemia as a Roadmap for Bone Marrow Failure: An Overview and a Clinical Workflow |
| title_fullStr | Aplastic Anemia as a Roadmap for Bone Marrow Failure: An Overview and a Clinical Workflow |
| title_full_unstemmed | Aplastic Anemia as a Roadmap for Bone Marrow Failure: An Overview and a Clinical Workflow |
| title_short | Aplastic Anemia as a Roadmap for Bone Marrow Failure: An Overview and a Clinical Workflow |
| title_sort | aplastic anemia as a roadmap for bone marrow failure an overview and a clinical workflow |
| topic | hematopoietic stem cells bone marrow immune-microenvironment bone marrow failure cytopenia aplastic anemia |
| url | https://www.mdpi.com/1422-0067/23/19/11765 |
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