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Start free with EleplanAcute myeloid leukemia and myelodysplastic syndromes related to topoisomerase type 2 inhibitor
ORPHA:102381Disease
Also called AML and myelodysplastic syndromes related to topoisomerase type 2 inhibitor
What it is
A subgroup of therapy-related myeloid neoplasms (t-MN), associated with treatment of an unrelated neoplastic disease with cytotoxic agents, like etoposid, doxorubicin, daunorubicin and others. The neoplastic cells often show rearrangements involving the mixed lineage leukemia gene at 11q23. This subgroup of t-MN is typically associated with overt leukemia, without preceding myelodysplastic syndrome, developing 2-3 years after exposure, presenting with non-specific symptoms related to ineffective hematopoesis (fatigue, bleeding and bruising, recurrent infections, bone pain) and/or extramedullary site involvement.
Key facts
- Classified as
- Disease
Recorded for the broader condition
- Prevalence
- <1 / 1 000 000 (annual incidence, Europe)Therapy related acute myeloid leukemia and myelodysplastic syndrome
- Age of onset
- All agesTherapy related acute myeloid leukemia and myelodysplastic syndrome
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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