Pathogenesis of Acquired Aplastic Anemia and the Role of the Bone Marrow Microenvironment
Aplastic anemia (AA) is characterized by bone marrow (BM) hypocellularity, resulting in peripheral cytopenias. An antigen-driven and likely auto-immune dysregulated T-cell homeostasis results in hematopoietic stem cell injury, which ultimately leads to the pathogenesis of the acquired form of this d...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2018-12-01
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| Series: | Frontiers in Oncology |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/article/10.3389/fonc.2018.00587/full |
| _version_ | 1818689098238394368 |
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| author | Michael Medinger Michael Medinger Beatrice Drexler Claudia Lengerke Jakob Passweg |
| author_facet | Michael Medinger Michael Medinger Beatrice Drexler Claudia Lengerke Jakob Passweg |
| author_sort | Michael Medinger |
| collection | DOAJ |
| description | Aplastic anemia (AA) is characterized by bone marrow (BM) hypocellularity, resulting in peripheral cytopenias. An antigen-driven and likely auto-immune dysregulated T-cell homeostasis results in hematopoietic stem cell injury, which ultimately leads to the pathogenesis of the acquired form of this disease. Auto-immune and inflammatory processes further influence the disease course as well as response rate to therapy, mainly consisting of intensive immunosuppressive therapy and allogeneic hematopoietic cell transplantation. Bone marrow hematopoietic stem and progenitor cells are strongly regulated by the crosstalk with the surrounding microenvironment and its components like mesenchymal stromal cells, also consistently altered in AA. Whether latter is a contributing cause or rather consequence of the disease remains an open question. Overall, niche disruption may contribute to disease progression, sustain pancytopenia and promote clonal evolution. Here we review the existing knowledge on BM microenvironmental changes in acquired AA and discuss their relevance for the pathogenesis and therapy. |
| first_indexed | 2024-12-17T12:04:42Z |
| format | Article |
| id | doaj.art-1c0c80331c4546aaad66d932892e8e9b |
| institution | Directory Open Access Journal |
| issn | 2234-943X |
| language | English |
| last_indexed | 2024-12-17T12:04:42Z |
| publishDate | 2018-12-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Oncology |
| spelling | doaj.art-1c0c80331c4546aaad66d932892e8e9b2022-12-21T21:49:41ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2018-12-01810.3389/fonc.2018.00587414997Pathogenesis of Acquired Aplastic Anemia and the Role of the Bone Marrow MicroenvironmentMichael Medinger0Michael Medinger1Beatrice Drexler2Claudia Lengerke3Jakob Passweg4Division of Internal Medicine, Department of Medicine, University Hospital Basel, Basel, SwitzerlandDivision of Hematology, Department of Medicine, University Hospital Basel, Basel, SwitzerlandDivision of Hematology, Department of Medicine, University Hospital Basel, Basel, SwitzerlandDivision of Hematology, Department of Medicine, University Hospital Basel, Basel, SwitzerlandDivision of Hematology, Department of Medicine, University Hospital Basel, Basel, SwitzerlandAplastic anemia (AA) is characterized by bone marrow (BM) hypocellularity, resulting in peripheral cytopenias. An antigen-driven and likely auto-immune dysregulated T-cell homeostasis results in hematopoietic stem cell injury, which ultimately leads to the pathogenesis of the acquired form of this disease. Auto-immune and inflammatory processes further influence the disease course as well as response rate to therapy, mainly consisting of intensive immunosuppressive therapy and allogeneic hematopoietic cell transplantation. Bone marrow hematopoietic stem and progenitor cells are strongly regulated by the crosstalk with the surrounding microenvironment and its components like mesenchymal stromal cells, also consistently altered in AA. Whether latter is a contributing cause or rather consequence of the disease remains an open question. Overall, niche disruption may contribute to disease progression, sustain pancytopenia and promote clonal evolution. Here we review the existing knowledge on BM microenvironmental changes in acquired AA and discuss their relevance for the pathogenesis and therapy.https://www.frontiersin.org/article/10.3389/fonc.2018.00587/fullaplastic anemiamicroenvironmentmicrovessel densitymesenchymal stem cellsstem cell niche |
| spellingShingle | Michael Medinger Michael Medinger Beatrice Drexler Claudia Lengerke Jakob Passweg Pathogenesis of Acquired Aplastic Anemia and the Role of the Bone Marrow Microenvironment Frontiers in Oncology aplastic anemia microenvironment microvessel density mesenchymal stem cells stem cell niche |
| title | Pathogenesis of Acquired Aplastic Anemia and the Role of the Bone Marrow Microenvironment |
| title_full | Pathogenesis of Acquired Aplastic Anemia and the Role of the Bone Marrow Microenvironment |
| title_fullStr | Pathogenesis of Acquired Aplastic Anemia and the Role of the Bone Marrow Microenvironment |
| title_full_unstemmed | Pathogenesis of Acquired Aplastic Anemia and the Role of the Bone Marrow Microenvironment |
| title_short | Pathogenesis of Acquired Aplastic Anemia and the Role of the Bone Marrow Microenvironment |
| title_sort | pathogenesis of acquired aplastic anemia and the role of the bone marrow microenvironment |
| topic | aplastic anemia microenvironment microvessel density mesenchymal stem cells stem cell niche |
| url | https://www.frontiersin.org/article/10.3389/fonc.2018.00587/full |
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