Anaplastic large cell lymphoma

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Anaplastic large cell lymphoma

ORPHA:98841Disease

Also called ALCL · CD30 positive anaplastic large cell lymphoma · Ki-1 positive anaplastic large cell lymphoma · Primary systemic ALCL · sACL

What it is

A rare and aggressive peripheral T-cell non-Hodgkin lymphoma, belonging to the group of CD30-positive lymphoproliferative disorders, which affects lymph nodes and extranodal sites. It is comprised of two sub-types, based on the expression of a protein called anaplastic lymphoma kinase (ALK): ALK positive and ALK negative ALCL.

Key facts

Prevalence
1-9 / 100 000 (Europe)
Age of onset
All ages
Inheritance
Not applicable
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Genes reported in subtypes

ALK

Orphanet records this gene on 1 more specific entry under this disorder, not on this entry itself:

A well-studied disease is usually split into subtypes, and the gene is curated on whichever one the evidence belongs to. Open a subtype to see which gene goes with it.

ICD-10 codes

C84.6ICD-10 uses a narrower term — shared with 1 other rare disease
C84.7ICD-10 uses a narrower term — shared with 2 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 3112MESH D017728MONDO 0020325UMLS C0206180

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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