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
Primary site must be bone marrow (C421)

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Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
See abstractor notes
Alternate Names
Acute myeloid leukemia, PML-RAR-alpha
Acute myeloid leukemia, t(11;17)(q23;q12)
Acute myeloid leukemia, t(15;17)(q22;q11-12)
Acute progranulocytic leukemia
Acute promyelocytic leukemia, NOS
Acute promyelocytic leukemia, PML-RAR-alpha
Acute promyelocytic leukemia, t(15;17)(q22;q11-12)
AML with a variant RARA translocation
APL
FAB M3 (includes all variants)
Hypergranular promyelocytic leukemia
PML-RARA
Definition
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.
Abstractor Notes
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. There may also be bleeding from venipuncture and bone marrow sites. The hemorrhagic diathesis may precede the diagnosis of leukemia by 2-8 weeks.

If the leukemia occurs before or simultaneously with Myeloid Sarcoma (9930/3), see M3 and Module 5:PH10.
Definitive Diagnostic Methods
Bone marrow biopsy
Cytochemistry
Genetic testing
Immunophenotyping
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 (ATRA)
Transformations to
None
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
Easy bruising or bleeding
Disseminated intravascular coagulation (DIC)
Fatigue
Fever
Hematuria
Menometrorrhagia (excessive irregular menstrual bleeding)
Petechiae
Rapidly rising leukocyte count
Shortness of breath
Weakness
Weight loss or loss of appetite
Diagnostic Exams
Recurrence and Metastases
CD56 is associated with less favorable prognosis
Epidemiology and Mortality
Age: predominantly adults in mid-life
Incidence: 5-8% of all AML cases
Survival: favorable when treated wotj ATRA