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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)
Per the WHO Blue Book, a diagnosis of Chronic myeloid leukemia with the BCR::ABL1 unspecified, code 9875/3 should be used instead of 9863/3. This is because most of the CML cases are BCR::ABL1 positive. CML BCR::ABL1 negative cases are rare and have additional testing and will be documented clearly.
Chronic myeloid leukemia, BCR::ABL1 negative is 9876/3.
So, do not use 9863/3 if the diagnosis is Chronic Myeloid Leukemia and there is no BCR::ABL1 status. Code 9875/3 to indicate CML, BCR::ABL1.
For CML BCR::ABL1 positive/unspecified, 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.
Per the WHO Blue Book, a diagnosis of Chronic myeloid leukemia with the BCR::ABL1 unspecified, code 9875/3 should be used instead of 9863/3. This is because most of the CML cases are BCR::ABL1 positive. CML BCR::ABL1 negative cases are rare and have additional testing and will be documented clearly.
Chronic myeloid leukemia, BCR::ABL1 negative is 9876/3.
So, do not use 9863/3 if the diagnosis is Chronic Myeloid Leukemia and there is no BCR::ABL1 status. Code 9875/3 to indicate CML, BCR::ABL1.
For CML BCR::ABL1 positive/unspecified, 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
Assign code 1 for histological confirmation WITH or WITHOUT positive immunophenotyping or genetics
Module Rule
None
Alternate Names
Chronic granulocytic leukemia, BCR::ABL1 (ICD-O-4: 98750/3)
Chronic granulocytic leukemia, Philadelphia chromosome, (Ph1) positive
Chronic granulocytic leukemia, t(9;22)(q34;q11) (ICD-O-4: 98750/3)
Chronic myelogenous leukemia, Philadelphia chromosome, (Ph1) positive
Chronic myelogenous leukemia, t(9;22)(q34;q11) (ICD-O-4: 98750/3)
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
Immunophenotyping
CD11b, CD13, CD15, CD33, CD64, CD65, CD117, CD177, MPO
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
