ALK-Positive Histiocytosis

Preferred Label : ALK-Positive Histiocytosis;

NCIt definition : A rare histiocytic neoplasm associated with ALK gene rearrangement leading to activation of signaling pathways. The neoplastic cells have a mature histiocytic phenotype, and usually have a foamy cytoplasm. It is characterized by frequent neurologic involvement and responds to ALK inhibition treatments.;

Neoplastic status : Undetermined;

NCI Metathesaurus CUI : CL1906947;

Details

Origin ID

C200105;

Has associated anatomic sites
- Hematopoietic and Lymphatic System [NCIt concept]
Has cytogenetic abnormalities
- ALK Gene Rearrangement [NCIt concept]
disease_excludes_finding
- CD30-Positive Neoplastic Cells Present [NCIt concept]
- HMB-45-Positive Neoplastic Cells Present [NCIt concept]
disease_excludes_molecular_abnormality
- Clonal Immunoglobulin Gene Rearrangement [NCIt concept]
- Clonal T-Cell Receptor Gene Rearrangement [NCIt concept]
disease_has_abnormal_cell
- Neoplastic Cell [NCIt concept]
- Neoplastic Hematopoietic and Lymphoid Cell [NCIt concept]
- Neoplastic Histiocytic and Dendritic Cell [NCIt concept]
disease_has_finding
- Histiocytosis [NCIt concept]
- Rare Lesion [NCIt concept]
disease_has_normal_cell_origin
- Hematopoietic and Lymphoid Cell [NCIt concept]
- Histiocyte [NCIt concept]
- Histiocytic and Dendritic Cell [NCIt concept]
disease_has_normal_tissue_origin
- Hematopoietic and Lymphoid Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Hematopoietic and Lymphatic System [NCIt concept]
disease_mapped_to_gene
- ALK Gene [NCIt concept]
pathogenesis_of_disease_involves_gene
- ALK Gene [NCIt concept]
- ALK wt Allele [NCIt concept]
- ALKATI Allele [NCIt concept]

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