Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
Preferred Name

Acute basophilic leukemia

Synonyms
Definitions

A rare acute myeloid leukemia characterized by primary differentiation to basophils. Microscopically, peripheral blood and bone marrow blasts contain coarse cytoplasmic basophilic granules which are positive with metachromatic staining (toluidine blue). Electron microscopy confirms that granules show features characteristic of basophil precursors. Mature basophils are usually sparse. Patients may present with manifestations related to bone marrow failure, as well as hepatosplenomegaly, cutaneous involvement, lytic lesions, and hyperhistaminemia. The disease is associated with a poor prognosis.

ID

http://www.orpha.net/ORDO/Orphanet_86849

definition

A rare acute myeloid leukemia characterized by primary differentiation to basophils. Microscopically, peripheral blood and bone marrow blasts contain coarse cytoplasmic basophilic granules which are positive with metachromatic staining (toluidine blue). Electron microscopy confirms that granules show features characteristic of basophil precursors. Mature basophils are usually sparse. Patients may present with manifestations related to bone marrow failure, as well as hepatosplenomegaly, cutaneous involvement, lytic lesions, and hyperhistaminemia. The disease is associated with a poor prognosis.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=86849

has_age_of_onset

All ages

hasDbXref

ICD-11:2A60.37

ICD-10:C94.7

MeSH:D015471

UMLS:C0023437

label

Acute basophilic leukemia

notation

ORPHA:86849

part_of

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

prefixIRI

ORDO:Orphanet_86849

prefLabel

Acute basophilic leukemia

present_in

Europe AND has_annual_incidence_range : <1 / 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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