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ALK-positive anaplastic large cell lymphoma
Disease definition
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).
ORPHA:300895
Classification level: Subtype of disorder- Synonym(s):
- ALK+ ALCL
- ALK+ anaplastic large cell lymphoma
- Prevalence: Unknown
- Inheritance: Not applicable
- Age of onset: Childhood, Adolescent, Adult
- ICD-10: C84.6
- OMIM: -
- UMLS: -
- MeSH: -
- GARD: -
- MedDRA: -
Summary
Epidemiology
The prevalence of ALK+ ALCL is unknown. This subtype usually affects children and young adults.
Clinical description
ALK+ ALCL is characterized by peripheral, mediastinal, or abdominal lymph node involvement. It manifests with the development of painless, enlarged lymph nodes, especially in the neck or armpit (axillary lymph nodes). General symptoms include loss of appetite and fatigue as well as fever, weight loss, and night sweats (B symptoms). Mediastinal involvement manifests as cough, dypsnea and/or edema. ALK+ ALCL can also extend to extranodal sites such as the bones, bone marrow, subcutaneous tissue, lungs, spleen and liver. The 5-year survival rate of ALK-positive patients is 70-80%.
Etiology
In ALK+ ALCL, the anaplastic lymphoma receptor tyrosine kinase ALK>/i> gene is overexpressed due to a t(2;5) (p23;q35) translocation.
Detailed information
Professionals
- Summary information
- Russian (2014, pdf)
- Clinical practice guidelines
- English (2011)
Additional information