raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:102381

Acute myeloid leukemia and myelodysplastic syndromes related to topoisomerase type 2 inhibitor

Synonyms: AML and myelodysplastic syndromes related to topoisomerase type 2 inhibitor

Type
Disease
Group
Disorder
Status
active

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.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enAcute myeloid leukemia and myelodysplastic syndromes related to topoisomerase type 2 inhibitorAML and myelodysplastic syndromes related to topoisomerase type 2 inhibitor
frLeucémie aiguë myéloïde et syndromes myélodysplasiques liés aux inhibiteurs de la topoisomérase II
deAkute myeloische Leukämie und myelodysplastische Syndrome durch Topoisomerase Typ II-InhibitorAML und MDS durch Topoisomerase Typ II-Inhibitor
esLeucemia mieloide aguda y síndromes mielodisplásicos relacionados con la topoisomerasa tipo 2 inhibidoraLMA y síndromes mielodisplásicos asociados al inhibidor de la topoisomerasa tipo 2
itLeucemia mieloide acuta e sindromi mielodisplastiche correlate all'inibitore della topoisomerasi tipo 2AML e sindromi mielodisplastiche associate all'inibitore della topoisomerasi tipo 2
nlAcute myeloïde leukemie en myelodysplastische syndromen gerelateerd aan topoisomerase type 2-remmerAML en myelodysplastische syndromen gerelateerd aan topoisomerase type 2-remmer
ptLeucemia mielóide aguda e síndromes mielodisplásticos relacionados inibidores das topoisomerases tipo II
plOstra białaczka szpikowa i zespoły mielodysplastyczne związane z inhibitorem topoizomerazy typu 2AML i zespoły mielodysplastyczne związane z inhibitorem topoizomerazy typu 2
csAkutní myeloidní leukémie a myelodysplastický syndrom související s inhibitorem topoisomerázy typu 2AML a myelodysplastický syndrom související s inhibitorem topoisomerázy typu 2
ukГострий мієлоїдний лейкоз та мієлодиспластичні синдроми, повязані з інгібітором топоізомерази 2 типуГМЛ та мієлодиспластичні синдроми, повязані з інгібітором топоізомерази типу 2
zh拓扑异构酶2型抑制剂相关急性髓系白血病及骨髓增生异常综合征与拓扑异构酶2型抑制物相关的急性髓系白血病和骨髓增生异常综合征
trTopoizomeraz tip 2 inhibitörüne bağlı akut miyeloid lösemi ve miyelodisplastik sendromlarTopoizomeraz tip 2 inhibitörü-ilişkili AML ve miyelodisplastik sendromlar

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10C92.0NTBTattributedvalidated
ICD-112A60.20NTBTindexvalidated
MONDO0015165Evalidated
UMLSC4707659Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card