Name

Acute promyelocytic leukemia with PML-RARA

ICD-O-1 Morphology

9866/3: Acute promyelocytic leukemia (AML with t(15;17)(q22;q12)) PML/RARA
Effective 1978 - 1991

ICD-O-2 Morphology

9866/3: Acute promyelocytic leukemia (AML with t(15;17)(q22;q12)) PML/RARA
Effective 1992 - 2000

ICD-O-3 Morphology

9866/3: Acute promyelocytic leukemia (AML with t(15;17)(q22;q12)) PML/RARA
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Grade

Not Applicable

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 with t(15;17)(q22;q12); PML-RARA
Acute promyelocytic leukemia, t(15;17)(q22;q11-12)
AML with a variant RARA translocation
APL
APL with PML-RARA
FAB M3 (includes all variants)
Hypergranular APL
Microgranular APL
PML-RARA

Definition

Acute promyelocytic leukemia (APL) with PML-RARA is an acute myeloid leukemia (AML) in which abnormal promyelocytes predominate. Both hypergranular (so-called typical) APL and microgranular (hypogranular) types exist.

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

FLT3
FLT3-ITD
FLT3-TKD (tyrosine kinase domain)
RARA gene on 17q21.2 fuses with nuclear regulatory gene on 15q24.1
Tretinoin (all-trans retinoic acid)

Immunophenotyping

CD2 expression
CD11a low or absent (hypergranular APL)
CD11b low or absent (hypergranular APL)
CD11c expression
CD13 bright expression (hypergranular APL)
CD15 weak or negative
CD18 low or absent (hypergranular APL)
CD33 bright expression (hypergranular APL)
CD34 low or absent (hypergranular APL)
CD56 expression
CD64 expression (microgranular APL)
CD65 weak or negative
HLA-DR low or absent (hypergranular APL)
KIT (CD117) expression
PML-RARA fusion gene

Treatments

Chemotherapy
Other therapy

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 (U.S. only)

C92.4 Acute promyelocytic leukemia (effective October 01, 2015)

Signs and Symptoms

Easy bruising or bleeding
Disseminated intravascular coagulation (DIC)
Menometrorrhagia (excessive irregular menstrual bleeding)
Rapidly rising leukocyte count
Shortness of breath
Weakness
Weight loss or loss of appetite

Progression and Transformation

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

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Acute myeloid leukemia and related precursor neoplasms
Pages: 134-136

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Acute Myeloid Leukemia
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Glossary