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ICD-O-3 Morphology
9875/3: Chronic myelogenous leukemia, BCR/ABL1 positive
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
Chronic myeloid leukemia (CML), BCR::ABL1 positive is part of the Myeloproliferative neoplasms (MPN) lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B2)
Most patients are diagnosed in the chronic phase. CML may be diagnosed when a blood count is performed as part of a routine medical examination.
Without treatment, most patients will progress to the blast phase within a couple of years. Progression to the blast phase is still the same primary.
- May also be documented as transformation of chronic phase to blast phase.
Diagnostic confirmation is usually confirmed with the genetics of t(9;22)(q34.1;q11.2) or positive BCR::ABL1.
- If BCR::ABL1 is negative, see 9876/3.
Immunophenotyping is used primarily to assess if the patient has gone into blastic phase. There are two blasts phases: myeloid and lymphoblastic.
Per the NCI PDQ, treatment for CML includes
* Targeted therapy with specific inhibitors of the BCR::ABL1 tyrosine kinase
* Other targeted therapy with tyrosine kinase inhibitors (TKI)
* Bone marrow or stem cell transplant
Splenectomy may also be done.
Patients with chronic myeloid leukemia (CML) may acquire MECOM rearrangement (see 9869); such cases are defined as blast phase regardless of the number of blasts. Although rare, cases with concurrent BCR::ABL1 and MECOM rearrangement at presentation are best regarded as blast phase CML.
Most patients are diagnosed in the chronic phase. CML may be diagnosed when a blood count is performed as part of a routine medical examination.
Without treatment, most patients will progress to the blast phase within a couple of years. Progression to the blast phase is still the same primary.
- May also be documented as transformation of chronic phase to blast phase.
Diagnostic confirmation is usually confirmed with the genetics of t(9;22)(q34.1;q11.2) or positive BCR::ABL1.
- If BCR::ABL1 is negative, see 9876/3.
Immunophenotyping is used primarily to assess if the patient has gone into blastic phase. There are two blasts phases: myeloid and lymphoblastic.
Per the NCI PDQ, treatment for CML includes
* Targeted therapy with specific inhibitors of the BCR::ABL1 tyrosine kinase
* Other targeted therapy with tyrosine kinase inhibitors (TKI)
* Bone marrow or stem cell transplant
Splenectomy may also be done.
Patients with chronic myeloid leukemia (CML) may acquire MECOM rearrangement (see 9869); such cases are defined as blast phase regardless of the number of blasts. Although rare, cases with concurrent BCR::ABL1 and MECOM rearrangement at presentation are best regarded as blast phase CML.
Diagnostic Confirmation
This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.
Module Rule
None
Alternate Names
Chronic granulocytic leukemia, BCR::ABL
Chronic granulocytic leukemia, Philadelphia chromosome, (Ph1) positive
Chronic granulocytic leukemia, t(9;22)(q34;q11)
Chronic myelogenous leukemia, Philadelphia chromosome, (Ph1) positive [OBS]
Chronic myelogenous leukemia, t(9;22)(q34;q11)
Chronic myeloid leukemia, t(9;22)(q34;q11)
CML, BCR-ABL1 positive, Accelerated phase (AP)
CML, BCR-ABL1 positive, Chronic phase (CP)
Definition
"Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm (MPN) defined by the BCR::ABL1 fusion gene and characterized by neutrophilic granulocytosis." (WHO 5th edition)
There are two phases for CML
1. Chronic phase (BP): the neoplastic cells are mostly confined to the blood, bone marrow, spleen, and liver.
2. Blast phase (BP): The blasts can infiltrate any extramedullary site, with a predilection for the spleen, liver, lymph nodes, skin, and soft tissues
There are two phases for CML
1. Chronic phase (BP): the neoplastic cells are mostly confined to the blood, bone marrow, spleen, and liver.
2. Blast phase (BP): The blasts can infiltrate any extramedullary site, with a predilection for the spleen, liver, lymph nodes, skin, and soft tissues
Definitive Diagnostic Methods
Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping
Genetics Data
Gain of chromosome 8 or 9 (more commonly found in blast phase)
BCR::ABL1 positive (if negative, see 9876/3)
Isochromosome 17q (more commonly found in blastic phase)
Philadelphia chromosome, (Ph1) positive
t(9;22)(q34.1;q11.2)
Immunophenotyping
CD2, CD3, CD4, CD5, CD7, CD8 (T-cell related antigens) (lymphoblastic blast phase)
CD10, CD19, CD79a, PAX50 (B-cell related antigens) (lymphoblastic blast phase)
CD11b- (no expression/negative) (myeloid blast phase)
CD11c+ (expression/positive) (myeloid blast phase)
CD13+ (expression/positive) (myeloid blast phase)
CD14+ (expression/positive) (myeloid blast phase)
CD15+ (expression/positive) (myeloid blast phase)
CD33+ (expression/positive) (myeloid blast phase)
CD41+ (expression/positive) (myeloid blast phase)
CD61+ (expression/positive) (myeloid blast phase)
Glycophorin A and C+ (expression/positive) (myeloid blast phase)
KIT (CD117)+ (expression/positive) (myeloid blast phase)
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy
Surgery
Transformations to
Transformations from
None
Same Primaries
Corresponding ICD-10 Codes (Cause of Death codes only)
C92.1 Chronic myeloid leukemia
Corresponding ICD-10-CM Codes (U.S. only)
C92.1 Chronic myeloid leukemia, BCR/ABL-positive (effective October 01, 2015 - September 30, 2024)
C92.10 Chronic myeloid leukemia, BCR/ABL-positive, not having achieved remission (effective October 01, 2024)
C92.11 Chronic myeloid leukemia, BCR/ABL-positive, in remission (effective October 01, 2024)
C92.12 Chronic myeloid leukemia, BCR/ABL-positive, in relapse (effective October 01, 2024)
Signs and Symptoms
Abnormal white blood count
Anemia
Bleeding complications/thrombotic
Fatigue
Fever
Malaise
Night sweats
Progressive leukocytosis
Splenomegaly
Thrombocytopenia
Thrombocytosis
Weight loss
Diagnostic Exams
Progression and Transformation
Progression to blastic phase (same primary)
Epidemiology and Mortality
Age: 50-60 years median age
Incidence: 1-2 cases per 100,000 population
Sex: Slight male predominance
Survival: 2-3 years (median), 4 years with conventional chemotherapy
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: Myeloproliferative neoplasms
Pages: Part A: 28-33
Section: Myeloproliferative neoplasms
Pages: Part A: 28-33
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
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 Myeloproliferative Neoplasms Treatment. Bethesda, MD: National Cancer Institute. Updated <09/27/24>. Available at: https://www.cancer.gov/types/myeloproliferative/hp/myeloproliferative-neoplasms-treatment. Accessed <01/22/25>. [PMID: 26389291]
Section: Chronic Myeloid Leukemia Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/leukemia/hp/cml-treatment-pdq
Section: Chronic Myeloid Leukemia Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/leukemia/hp/cml-treatment-pdq
