Myelodysplastic syndrome and aplastic anemia: distinct entities or diseases linked by a common pathophysiology?
Abstract
It is often difficult to distinguish myelodysplastic syndrome (MDS) from severe aplastic anemia (SAA) because both can present with profoundly hypocellular bone marrows. The distinction matters because although both conditions are complicated by pancytopenia, the risk of progression to acute leukemia is much greater in MDS. This chapter reexamines the relationship between SAA and MDS. The clinical and morphological features and pathophysiology of AA (including moderate and severe forms of acquired AA) are compared with MDS and hypoplastic MDS, with particular reference to new observations implicating autoimmune processes in both conditions. SAA and hypoplastic MDS (HMDS) are discussed in the light of these findings and attempts to separate nonevolving bone marrow failure syndromes from marrow failure progressing to acute leukemia are reviewed. The weight of evidence supports a common pathophysiology and, more speculatively, a common etiology for at least some forms of AA and MDS.
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Hypocellular MDS can be understood as an exhausted form of AA in which selective clones have been established.
“Thus, hypocellular MDS can be understood as an exhausted form of AA in which selective clones have been established.65”
From Inflammatory bone marrow microenvironment (2019) · cites this paper as [65]
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- Authors
- Barrett, J., Saunthararajah, Y., & Molldrem, J.
- Affiliations
- National Institutes of Health (US)
- Corresponding author
- Barrett, John
- Venue
- Seminars in Hematology
- Publisher
- Elsevier BV
- Published
- 2000-01
- Volume
- 37
- Issue
- 1
- Pages
- 15-29
- ISSN
- 0037-1963 (ISSN-L)
- OpenAlex
- W2053333573
- Cited by
- 207
- References
- 95
- Citations by year
- 2026: 2 · 2025: 1 · 2024: 3 · 2023: 5 · 2022: 3 · 2021: 2 · 2020: 4 · 2019: 3 · 2018: 3 · 2017: 9 · 2016: 2 · 2015: 2 · 2014: 6 · 2013: 6 · 2012: 11
- FWCI
- 2.92
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Seminars in Hematology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Hematology › Acute Myeloid Leukemia Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Blood disorders and treatments
- Health Sciences › Medicine › Emergency Medicine › Hematological disorders and diagnostics
- Keywords
- myelodysplastic syndrome, severe aplastic anemia, hypoplastic myelodysplastic syndrome, bone marrow failure, pancytopenia, autoimmune processes, pathophysiology, disease classification, acquired aplastic anemia
- MeSH terms
- Anemia, Aplastic, Bone Marrow, Diagnosis, Differential, Humans, Immunosuppressive Agents, Myelodysplastic Syndromes, Pancytopenia, T-Lymphocytes
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