" /> Acute Promyelocytic Leukemia - CISMeF





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;

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31/07/2025


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