Acute promyelocytic leukemia with t(15;17)(q22;q12); PML-RARA

Name
Acute promyelocytic leukemia with t(15;17)(q22;q12); PML-RARA
ICD-O-1 Morphology
9866/3
Effective 1978 - 1991
ICD-O-2 Morphology
9866/3
Effective 1992 - 2000
ICD-O-3 Morphology
9866/3
Effective 2001 and later
Reportable
for cases diagnosed 1978 and later
Primary Site(s)
C421

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Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
M3 Module 5: PH10
Alternate Names
Acute myeloid leukemia, PML-RAR-alpha
Acute myeloid leukemia, t(15;17)(q22;q11-12)
Acute progranulocytic leukemia
Acute promyelocytic leukemia, t(15;17)(q22;q11-12)
Acute promyelocytic leukemia, NOS
Acute promyelocytic leukemia, PML-RAR-alpha
Acute promyelocytic leukemia, t(15;17)(q22;q11-12)
APL
FAB M3 (includes all variants)
Hypergranular promyelocytic leukemia
PML-RARA
Definition
Acute myeloid leukemia in which abnormal promyelocytes predominate
Abstractor Notes
Acute promyelocytic leukemia is a malignancy of the bone marrow in which there is a deficiency of mature blood cells in the myeloid line of cells and an excess of immature cells called promyelocytes. APL is due to a translocation (an exchange of chromosome material) between chromosomes 15 and 17 which is symbolized t(15;17). This translocation is not a mere marker of APL. It is the cause of APL. APL is consistently associated with a disorder that resembles (but is not identical to) disseminated intravascular coagulation (DIC). In APL, there is a pronounced tendency to hemorrhage. The bleeding can manifest itself as petechiae (little bleeding spots in the skin or elsewhere), small ecchymoses (bruises), epistaxis (nose bleeds), bleeding in the mouth, hematuria (blood in the urine), bleeding from venipuncture and bone marrow sites, and girls and women who are menstruating may have menometrorrhagia (excessive irregular menstrual bleeding). The hemorrhagic diathesis may precede the diagnosis of leukemia by 2-8 weeks.
Definitive Diagnostic Methods
Bone Marrow biopsy
Cytochemistry
Genetics Data
CD2
CD9
CD11a
CD13
CD15 negative
CD18
CD33
CD34 absent or low
Retinoic acid receptor alpha (RAR) on 17q12 fuses with 15q22 (PML gene)
Immunophenotyping
CD33
HLA-DR absent or low
PML-RARA fusion gene
Treatments
Chemotherapy
Other RX
Transformations to
No Transformations
Corresponding ICD-9 Codes
205.0 Acute myeloid leukemia
Corresponding ICD-10 Codes
C92.4 Acute promyelocytic leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C92.4 Acute promyelocytic leukemia
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None