raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:300895

ALK-positive anaplastic large cell lymphoma

Synonyms: ALK+ ALCL · ALK+ anaplastic large cell lymphoma

Type
Histopathological subtype
Group
Subtype of disorder
Inheritance
Not applicable
Age of onset
Adolescent, Adult, Childhood
Status
active

A type of ALCL, a rare and aggressive peripheral T-cell non-Hodgkin lymphoma affecting lymph nodes and extranodal sites, which is characterized by the expression of a protein called anaplastic lymphoma kinase (ALK).

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enALK-positive anaplastic large cell lymphomaALK+ ALCL · ALK+ anaplastic large cell lymphoma
frLymphome anaplasique à grandes cellules ALK positifALCL ALK+ · LAGC ALK+ · Lymphome anaplasique à grandes cellules ALK+
deLymphom, anaplastisches großzelliges, ALK-positivesALK+ ALCL · ALK+ anaplastisches großzelliges Lymphom
esLinfoma anaplásico de células grandes ALK-positivoALK+ ALCL · Linfoma anaplásico de células grandes ALK+
itLinfoma anaplastico a grandi cellule ALK-positivoALK+ ALCL · Linfoma anaplastico a grandi cellule ALK+
nlALK-positief anaplastisch grootcellig lymfoomALK+ ALCL · ALK+ anaplastisch grootcellig lymfoom
ptLinfoma anaplásico de grandes células ALK - positivo
plALK-pozytywny chłoniak anaplastyczny z dużych komórekALK+ ALCL · ALK+ chłoniak anaplastyczny z dużych komórek
csALK-pozitivní anaplastický velkobuněčný lymfomALK+ ALCL · ALK+ anaplastický velkobuněčný lymfom
ukALK-позитивна анапластична великоклітинна лімфомаALK+ ALCL · Анапластична великоклітинна лімфома ALK+
zhALK阳性间变性大细胞淋巴瘤ALK阳性间变性大细胞淋巴瘤
trALK-pozitif anaplastik büyük hücreli lenfomaALK+ ALCL · ALK + anaplastik büyük hücreli lenfoma

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10C84.6Especificvalidated
ICD-112A90.AEspecificvalidated
ICD-11XH9484Especificvalidated
MONDO0017602Evalidated
UMLSC1332079Evalidated

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

1 direct
GeneAssociationStatusHGNCEnsemblOMIMLocusNameSource of validation
ALKDisease-causing somatic mutation(s) inAssessedHGNC:427ENSG000001710941055902p23.2-p23.1ALK receptor tyrosine kinase12112524[PMID]
5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—EuropeClass onlyNot yet validatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

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

7

Sources for this card