Acute Promyelocytic Leukemia

Preferred Label : Acute Promyelocytic Leukemia;

NCIt synonyms : M3; APML; FAB M3;

NCIt related terms : promyelocytic leukemia; APL;

NCIt definition : An acute myeloid leukemia characterized by the predominance of abnormal promyelocytes and translocations involving the retinoic acid receptor-α (RARA) gene. Over 95% of cases are characterized by the presence of the reciprocal balanced translocation t(15;17)(q24.1;q21.2) that results in the fusion of the promyelocytic leukemia (PML) gene and RARA gene. The remainder of the cases show variant RARA gene translocations with other genes including NUMA1, ZBTB16, STAT5B, and NPM1.;

Alternative definition : NCI-GLOSS: An aggressive (fast-growing) type of acute myeloid leukemia in which there are too many immature blood-forming cells in the blood and bone marrow. It is usually marked by an exchange of parts of chromosomes 15 and 17.; NICHD: Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia (AML), and it is treated differently than other types of AML because of its marked sensitivity to differentiating effects of all trans-retinoic acid. APL is characterized by a severe coagulopathy which may be present at diagnosis. APL occurs in 7 % of children with AML.;

Neoplastic status : Malignant;

Codes from synonyms : 10001019; CDR0000444957; CDR0000046159; CDR0000522912;

Details

Origin ID

C208352;

Disease excludes abnormal cell
- Neoplastic Lymphoblast [NCIt concept]
Disease excludes normal cell origin
- Lymphocyte [NCIt concept]
Disease may have findings
- Hypercellular Bone Marrow [NCIt concept]
Has associated anatomic sites
- Bone Marrow [NCIt concept]
- Hematopoietic and Lymphatic System [NCIt concept]
Has cytogenetic abnormalities
- Chromosomal Rearrangement [NCIt concept]
- RARA Gene Rearrangement [NCIt concept]
concept_is_in_subset
- AML Authorized Value Terminology [NCIt concept]
- AML Disease Characteristics Table [NCIt concept]
- GDC Terminology [NCIt concept]
- GDC Value Terminology [NCIt concept]
- NCI CTEP SDC Acute Myeloid Leukemia Sub-Category Terminology [NCIt concept]
- NCI CTEP Simplified Disease Classification Terminology [NCIt concept]
- NCIt Neoplasm Core Terminology [NCIt concept]
- NICHD Terminology [NCIt concept]
- Pediatric Hematology-Oncology Terminology [NCIt concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Chronic Clinical Course [NCIt concept]
- Cytogenetic Abnormalities Not Detected [NCIt concept]
- Solid Growth Pattern [NCIt concept]
disease_has_abnormal_cell
- Leukemic Cell [NCIt concept]
- Malignant Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Hematopoietic and Lymphoid Cell [NCIt concept]
- Neoplastic Myeloblast [NCIt concept]
- Neoplastic Myeloid Cell [NCIt concept]
- Neoplastic Promyelocyte [NCIt concept]
disease_has_finding
- Blasts 10 Percent or More of Bone Marrow Nucleated Cells [NCIt concept]
- Blasts 10 Percent or More of Peripheral Blood White Cells [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Neoplastic Promyelocytes in Bone Marrow Markedly Increased [NCIt concept]
- Recurrent Genetic Abnormalities Present [NCIt concept]
disease_has_molecular_abnormality
- Fusion Protein Expression [NCIt concept]
disease_has_normal_cell_origin
- Bone Marrow Stem Cell [NCIt concept]
- Bone Marrow Stem Cell with Potential to Differentiate to Granulocytic Lineage [NCIt concept]
- Hematopoietic and Lymphoid Cell [NCIt concept]
disease_has_normal_tissue_origin
- Hematopoietic Tissue [NCIt concept]
- Hematopoietic and Lymphoid Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Bone Marrow [NCIt concept]
- Hematopoietic and Lymphatic System [NCIt concept]
disease_mapped_to_gene
- RARA Gene [NCIt concept]
disease_may_have_abnormal_cell
- Faggot Cell [NCIt concept]
disease_may_have_associated_disease
- Disseminated Intravascular Coagulation [NCIt concept]
pathogenesis_of_disease_involves_gene
- RARA Gene [NCIt concept]
- RARA wt Allele [NCIt concept]

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