Search Database ICD-O-3 Code Lists

Name

Acute promyelocytic leukemia with PML::RARA fusion (APL with PML::RARA)

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)

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Acute promyelocytic leukemia with PML::RARA fusion (APL with PML::RARA) is part of the Acute myeloid leukemia (AML) lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B6)

The peripheral blood and bone marrow are the principle sites of involvement. Rare cases have extramedullary involvement.

If this leukemia and myeloid sarcoma (9930/3) occur during the same clinical workup, this is one primary, the leukemia.

If the myeloid sarcoma occurs after the diagnosis of the leukemia, that is a manifestation of the leukemia and is the same primary.

See Multiple Primary Rule M3

Diagnostic Confirmation

This AML is part of the "AML with recurrent genetic abnormalities" group. Since this AML is diagnosed based on genetics, diagnostic confirmation will always be 3.

Module Rule

See abstractor notes

Alternate Names

Acute myeloid leukemia, PML/RAR-alpha
Acute myeloid leukemia, t(15;17)(q22;q11-12)
Acute promyelocytic leukemia, PML/RAR-alpha
Acute promyelocytic leukemia with t(15;17)(q22;q12); PML-RARA
FAB M3 (includes all variants)

Definition

Acute promyelocytic leukemia (APL) with PML::RARA is a type of acute myeloid leukemia (AML), characterized by a predominance of abnormal promyelocytes and fusion of the promyelocytic leukemia gene (PML) with the retinoic acid receptor alpha gene (RARA). (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing

Genetics Data

PML::RARA
t(15;17)(q22;q11-12)

Immunophenotyping

Immunophenotyping not relevant, diagnosed via genetic analysis

Treatments

Chemotherapy
Other therapy

Transformations to

None

Transformations from

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C92.4 Acute promyelocytic leukemia

Corresponding ICD-10-CM Codes (U.S. only)

C92.4 Acute promyelocytic leukemia (effective October 01, 2015 - September 30, 2024)
C92.40 Acute promyelocytic leukemia, not having achieved remission (effective October 01, 2024)
C92.41 Acute promyelocytic leukemia, in remission (effective October 01, 2024)
C92.42 Acute promyelocytic leukemia, in relapse (effective October 01, 2024)

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

Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunophenotyping
Lumbar puncture
Molecular analysis
Peripheral blood smear
Physical exam and history

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

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Acute myeloid leukemia
Pages: Part A: 112-114

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

PDQ® Adult Treatment Editorial Board. PDQ Acute Myeloid Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <03/06/2024>. Available at: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq. Accessed <02/06/2025>. [PMID: 26389432]
Section: Acute Myeloid Leukemia Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Glossary