ALK-positive anaplastic large cell lymphoma

GPTKB entity

Statements (51)
Predicate Object
gptkbp:instanceOf gptkb:disease
leukemia
gptkbp:causedBy gptkb:chromosomal_translocation_t(2;5)(p23;q35)
gptkbp:firstDescribed 1985
gptkbp:frequency more common in males
accounts for 10-15% of childhood non-Hodgkin lymphomas
accounts for 2-3% of adult non-Hodgkin lymphomas
gptkbp:hasBiomarker ALK gene rearrangement
NPM-ALK fusion protein
gptkbp:hasGeneticAlteration gptkb:A20-ALK_fusion
gptkb:ATIC-ALK_fusion
gptkb:CLTC-ALK_fusion
gptkb:MSN-ALK_fusion
gptkb:MYH9-ALK_fusion
gptkb:NPM1-ALK_fusion
gptkb:NPM1-ALK_fusion_protein
gptkb:RNF213-ALK_fusion
gptkb:TPM3-ALK_fusion
gptkb:TPR-ALK_fusion
gptkb:TRAF1-ALK_fusion
gptkbp:hasImmunophenotype CD30 positive
ALK positive
CD3 negative or variable
EMA positive
gptkbp:hasOrphanetID ORPHA:98256
gptkbp:hasRelapseRisk relapse possible after treatment
gptkbp:hasTypicalAgeOfOnset children
young adults
gptkbp:histology horseshoe-shaped nuclei
large anaplastic cells
https://www.w3.org/2000/01/rdf-schema#label ALK-positive anaplastic large cell lymphoma
gptkbp:ICD-10_code C84.6
gptkbp:originatedIn gptkb:T-cell
gptkbp:prevalence rare
gptkbp:prognosis generally favorable in children and young adults
worse prognosis in older adults
gptkbp:relatedTo gptkb:ALK-negative_anaplastic_large_cell_lymphoma
gptkb:primary_cutaneous_anaplastic_large_cell_lymphoma
gptkbp:stageName gptkb:Ann_Arbor_staging_system
gptkbp:subspecies gptkb:primary_cutaneous_ALK-positive_anaplastic_large_cell_lymphoma
gptkb:systemic_ALK-positive_anaplastic_large_cell_lymphoma
gptkbp:symptom fever
weight loss
lymphadenopathy
night sweats
gptkbp:treatment gptkb:brentuximab_vedotin
chemotherapy
stem cell transplant
gptkbp:WHOClassification gptkb:mature_T-cell_and_NK-cell_neoplasm
gptkbp:bfsParent gptkb:anaplastic_large_cell_lymphoma
gptkbp:bfsLayer 6