Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
Preferred Name

Atypical chronic myeloid leukemia
Synonyms

Subacute myeloid leukemia

Definitions

A rare myelodysplastic/myeloproliferative neoplasm characterized by peripheral blood leukocytosis due to increased numbers of morphologically dysplastic neutrophils and their precursors, hypercellular bone marrow with granulocytic proliferation and dysplasia (with or without dysplasia in the erythroid and megakaryocytic lineages), and prominent dysgranulopoiesis, but no or minimal absolute basophilia or monocytosis. Blasts account for less than 20% of leukocytes in the blood and bone marrow. BCR-ABL1 fusion is absent, as well as PDGFRA, PDGFRB or FGFR1 rearrangement, or PCM1-JAK2. Patients may present with signs and symptoms related to splenomegaly, anemia, or thrombocytopenia. Prognosis is generally poor.

ID

http://www.orpha.net/ORDO/Orphanet_98824

alternative_term

Subacute myeloid leukemia

definition

A rare myelodysplastic/myeloproliferative neoplasm characterized by peripheral blood leukocytosis due to increased numbers of morphologically dysplastic neutrophils and their precursors, hypercellular bone marrow with granulocytic proliferation and dysplasia (with or without dysplasia in the erythroid and megakaryocytic lineages), and prominent dysgranulopoiesis, but no or minimal absolute basophilia or monocytosis. Blasts account for less than 20% of leukocytes in the blood and bone marrow. BCR-ABL1 fusion is absent, as well as PDGFRA, PDGFRB or FGFR1 rearrangement, or PCM1-JAK2. Patients may present with signs and symptoms related to splenomegaly, anemia, or thrombocytopenia. Prognosis is generally poor.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=98824

has_age_of_onset

Adult

hasDbXref

UMLS:C1292772

MeSH:D054438

ICD-10:C92.2

ICD-11:2A41

MedDRA:10054651

label

Atypical chronic myeloid leukemia

notation

ORPHA:98824

part_of

http://www.orpha.net/ORDO/Orphanet_98275

prefixIRI

ORDO:Orphanet_98824

prefLabel

Atypical chronic myeloid leukemia

present_in

Europe AND has_annual_incidence_range : <1 / 1 000 000

treeView

http://www.orpha.net/ORDO/Orphanet_98275

subClassOf

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

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

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