Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
Preferred Name

Acute myelomonocytic leukemia
Synonyms

AMMoL

AML M4

Definitions

A rare acute myeloid leukemia disorder characterized by increased blast cells (myeloblasts, monoblast, and/or promonoblasts), representing more than 20% of the total bone marrow (BM) or peripheral blood differential counts, with 20-80% of BM cells being of monocytic lineage. Clinical presentation is the result of bone marrow involvement and extramedullary infiltration by the leukemic cells and includes asthenia, pallor, fever, dizziness, respiratory symptoms, easy bruising, bleeding disorders, and neurological deficits. Gingival hyperplasia, organomegaly, especially hepatosplenomegaly, and lymphadenopathy may also be associated.

ID

http://www.orpha.net/ORDO/Orphanet_517

alternative_term

AMMoL

AML M4

definition

A rare acute myeloid leukemia disorder characterized by increased blast cells (myeloblasts, monoblast, and/or promonoblasts), representing more than 20% of the total bone marrow (BM) or peripheral blood differential counts, with 20-80% of BM cells being of monocytic lineage. Clinical presentation is the result of bone marrow involvement and extramedullary infiltration by the leukemic cells and includes asthenia, pallor, fever, dizziness, respiratory symptoms, easy bruising, bleeding disorders, and neurological deficits. Gingival hyperplasia, organomegaly, especially hepatosplenomegaly, and lymphadenopathy may also be associated.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=517

has_age_of_onset

Adult

has_inheritance

Not applicable

hasDbXref

ICD-11:2A60.33

MedDRA:10000890

ICD-10:C92.5

MeSH:D015479

UMLS:C0023479

label

Acute myelomonocytic leukemia

notation

ORPHA:517

part_of

http://www.orpha.net/ORDO/Orphanet_167714

prefixIRI

ORDO:Orphanet_517

prefLabel

Acute myelomonocytic leukemia

present_in

Europe AND has_point_prevalence_range : 1-9 / 1 000 000

Europe AND has_annual_incidence_average_value : 0.17 AND has_annual_incidence_range : 1-9 / 1 000 000

treeView

http://www.orpha.net/ORDO/Orphanet_167714

subClassOf

http://www.orpha.net/ORDO/Orphanet_377788

http://www.orpha.net/ORDO/Orphanet_557493

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