Aggressive Systemic Mastocytosis

Preferred Label : Aggressive Systemic Mastocytosis;

NCIt synonyms : ASM; Advanced Systemic Mastocytosis;

NCIt definition : An aggressive and progressive mast cell neoplasm characterized by systemic infiltration of internal organs by aggregates of neoplastic mast cells. There is no evidence of mast cell leukemia or clonal hematologic malignancy. Clinical symptoms include hepatomegaly, splenomegaly, portal hypertension, malabsorption syndrome, and pathologic fractures.;

Neoplastic status : Malignant;

ICD-O code : 9741/3;

Codes from synonyms : C96.21;

Details

Origin ID

C9285;

UMLS CUI

C1112486;

Currated CISMeF NLP mapping
- Aggressive systemic mastocytosis [ORDO Disease]
- Aggressive systemic mastocytosis [MedDRA LLT]
- Aggressive systemic mastocytosis (disorder) [SNOMED CT concept]
- Aggressive systemic mastocytosis (morphologic abnormality) [SNOMED CT concept]
- aggressive systemic mastocytosis [DO Concept]
- aggressive systemic mastocytosis [Disease (Nov.)]
- aggressive systemic mastocytosis [MeSH concept]
DO Cross reference
- aggressive systemic mastocytosis [DO Concept]
Disease may have findings
- Cytopenia [NCIt concept]
- Hepatomegaly [NCIt concept]
- Hypersplenism [NCIt concept]
- Lytic Bone Lesion [NCIt concept]
- Malabsorption Syndrome [NCIt concept]
- Pathologic Fracture [NCIt concept]
- Portal Hypertension [NCIt concept]
- Serum Total Tryptase Levels Increased [NCIt concept]
- Splenomegaly [NCIt concept]
Has associated anatomic sites
- Hematopoietic and Lymphatic System [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- Aggressive lymphadenopathic mastocytosis with eosinophilia (disorder) [SNOMED CT concept]
- Aggressive systemic mastocytosis [MedDRA LLT]
- Aggressive systemic mastocytosis [ORDO Disease]
- Aggressive systemic mastocytosis (disorder) [SNOMED CT concept]
- Aggressive systemic mastocytosis (morphologic abnormality) [SNOMED CT concept]
- aggressive systemic mastocytosis [DO Concept]
- aggressive systemic mastocytosis [Disease (Nov.)]
- aggressive systemic mastocytosis [MeSH concept]
concept_is_in_subset
- CTRP Disease Terminology [NCIt concept]
- CTRP Terminology [NCIt concept]
- GDC Terminology [NCIt concept]
- GDC Value Terminology [NCIt concept]
- NCIt Neoplasm Core Terminology [NCIt concept]
- mCode Cancer Disorder Value Set [NCIt concept]
- mCode Elixhauser Lymphoma Value Set [NCIt concept]
- mCode Primary Cancer Disorder Value Set [NCIt concept]
- mCode Primary Malignant Neoplasm Disorder Value Set [NCIt concept]
- mCode Terminology [NCIt concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Mast Cells 10 Percent or More of Peripheral Blood White Cells [NCIt concept]
- Mast Cells 20 Percent or More of Bone Marrow Nucleated Cells [NCIt concept]
disease_has_abnormal_cell
- Malignant Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Hematopoietic and Lymphoid Cell [NCIt concept]
- Neoplastic Mast Cell [NCIt concept]
- Neoplastic Myeloid Cell [NCIt concept]
disease_has_finding
- Aggressive Clinical Course [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Monoclonal Cellular Population Present [NCIt concept]
- Primary Lesion [NCIt concept]
- Rare Lesion [NCIt concept]
- Systemic Disease [NCIt concept]
disease_has_normal_cell_origin
- Bone Marrow Stem Cell [NCIt concept]
- Hematopoietic and Lymphoid Cell [NCIt concept]
- Mast Cell [NCIt concept]
disease_has_normal_tissue_origin
- Hematopoietic Tissue [NCIt concept]
- Hematopoietic and Lymphoid Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Hematopoietic and Lymphatic System [NCIt concept]
disease_mapped_to_gene
- KIT Gene [NCIt concept]
disease_may_have_molecular_abnormality
- C-KIT Somatic Point Mutation [NCIt concept]
- C-KIT Tyrosine Kinase Protein Overexpression [NCIt concept]

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