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Name
Chronic myeloid leukemia, BCR-ABL1-positive
ICD-O-1 Morphology
9863/3: Chronic myeloid leukemia, NOS
Effective
1978 - 1991
ICD-O-2 Morphology
9863/3: Chronic myeloid leukemia, NOS
Effective
1992 - 2000
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
Diagnosis of this neoplasm is usually incidental (85% are asymptomatic when diagnosed) when the patient has a CBC and/or peripheral blood smear. If the results of the WBC are abnormal (elevated) the physician will order a bone marrow aspiration.
Treatment information:
Chronic phase:
Tyrosine kinase inhibitor; high-dose chemotherapy with donor cell transplant; BRM (interferon) with or without chemotherapy. May also have single or multi-agent chemotherapy and splenectomy.
Accelerated phase:
Donor stem cell transplant; tyrosine kinase inhibitor; BRM (interferon) with or without chemotherapy.
Blast phase:
Tyrosine kinase inhibitor; single or multi-drug chemotherapy; donor stem cell transplant.
Surgery includes splenectomy.
Aspirin was previously documented as treatment for this disease. This was found to be incorrect. Treatment has been updated based on the NCI website (updated 6/12/15)
Treatment information:
Chronic phase:
Tyrosine kinase inhibitor; high-dose chemotherapy with donor cell transplant; BRM (interferon) with or without chemotherapy. May also have single or multi-agent chemotherapy and splenectomy.
Accelerated phase:
Donor stem cell transplant; tyrosine kinase inhibitor; BRM (interferon) with or without chemotherapy.
Blast phase:
Tyrosine kinase inhibitor; single or multi-drug chemotherapy; donor stem cell transplant.
Surgery includes splenectomy.
Aspirin was previously documented as treatment for this disease. This was found to be incorrect. Treatment has been updated based on the NCI website (updated 6/12/15)
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.
Grade
Not Applicable
Module Rule
None
Alternate Names
Chronic granulocytic leukemia, BCR/ABL positive
Chronic granulocytic leukemia, BCR-ABL [OBS]
Chronic granulocytic leukemia, t(9;22)(q34;q11)
Chronic myelogenous leukemia, BCR/ABL positive
Chronic myelogenous leukemia, t(9;22)(q34;q11) [OBS]
Chronic myelogenous leukemia, t(9;22)(q34;q11.2) [OBS]
CML, BCR-ABL1 positive
CML, BCR-ABL1 positive, Accelerated phase (AP)
Definition
Chronic myeloid leukemia (CML), BCR-ABL1-positive, is a myeloproliferative neoplasm (MPN) in which granulocytes are the major proliferative component. It arises in a hematopoietic stem cell and is characterized by the chromosomal translocation t(9;22)(q34.1;q11.2), which results in the formation of the Philadelphia (Ph) chromosome, containing the BCR-ABL1 fusion gene.
CML has four phases:
1. Accelerated phase - can last weeks to months.
2. Chronic phase - involvement is usually limited to blood, bone marrow and spleen, although the liver may be infiltrated.
3. Blastic phase - lymph nodes and tissue may be involved. The blastic phase is a progression from the chronic phase. It remains the same histology: Chronic myelogenous leukemia.
4. Terminal phase - last phase and survival is usually only weeks or months
Criteria for CML, accelerated phase:
CML, accelerated phase criteria
Any 1 or more of the following hematologic/cytogenetic criteria or response-to-TKI criteria:
• Persistent or increasing WBC (.10 3 109/L), unresponsive to therapy “Provisional” response-to-TKI criteria
• Persistent or increasing splenomegaly, unresponsive to therapy
• Hematologic resistance to the first TKI (or failure to
achieve a complete hematologic response* to the first
TKI) or
• Persistent thrombocytosis (.1000 3 109/L), unresponsive to therapy • Any hematological, cytogenetic, or molecular indications
of resistance to 2 sequential TKIs or
• Persistent thrombocytopenia (,100 3 109/L) unrelated to therapy • Occurrence of 2 or more mutations in BCR-ABL1 during
TKI therapy
• 20% or more basophils in the PB
• 10%-19% blasts† in the PB and/or BM
• Additional clonal chromosomal abnormalities in Ph1 cells at diagnosis that include
“major route” abnormalities (second Ph, trisomy 8, isochromosome 17q, trisomy
19), complex karyotype, or abnormalities of 3q26.2
• Any new clonal chromosomal abnormality in Ph1 cells that occurs during therapy
CML has four phases:
1. Accelerated phase - can last weeks to months.
2. Chronic phase - involvement is usually limited to blood, bone marrow and spleen, although the liver may be infiltrated.
3. Blastic phase - lymph nodes and tissue may be involved. The blastic phase is a progression from the chronic phase. It remains the same histology: Chronic myelogenous leukemia.
4. Terminal phase - last phase and survival is usually only weeks or months
Criteria for CML, accelerated phase:
CML, accelerated phase criteria
Any 1 or more of the following hematologic/cytogenetic criteria or response-to-TKI criteria:
• Persistent or increasing WBC (.10 3 109/L), unresponsive to therapy “Provisional” response-to-TKI criteria
• Persistent or increasing splenomegaly, unresponsive to therapy
• Hematologic resistance to the first TKI (or failure to
achieve a complete hematologic response* to the first
TKI) or
• Persistent thrombocytosis (.1000 3 109/L), unresponsive to therapy • Any hematological, cytogenetic, or molecular indications
of resistance to 2 sequential TKIs or
• Persistent thrombocytopenia (,100 3 109/L) unrelated to therapy • Occurrence of 2 or more mutations in BCR-ABL1 during
TKI therapy
• 20% or more basophils in the PB
• 10%-19% blasts† in the PB and/or BM
• Additional clonal chromosomal abnormalities in Ph1 cells at diagnosis that include
“major route” abnormalities (second Ph, trisomy 8, isochromosome 17q, trisomy
19), complex karyotype, or abnormalities of 3q26.2
• Any new clonal chromosomal abnormality in Ph1 cells that occurs during therapy
Definitive Diagnostic Methods
Bone marrow biopsy
FISH
Genetic testing
Polymerase chain reaction (PCR)
Genetics Data
Immunophenotyping
CD2, CD3, CD4, CD5, CD7, CD8 (T-cell related antigens)
CD10, CD19, CD79a, PAX50 (B-cell related antigens)
CD11b- (no expression/negative)
CD11c+ (expression/positive)
CD13+ (expression/positive)
CD14+ (expression/positive)
CD15+ (expression/positive)
CD33+ (expression/positive)
CD34+ (expression/positive)
CD41+ (expression/positive)
CD56- (no expression/negative)
CD61+ (expression/positive)
Glycophorin A and C+ (expression/positive)
KIT (CD117)+ (expression/positive)
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy
Surgery
Transformations to
Transformations from
None
Same Primaries
Corresponding ICD-9 Codes
205.1 Chronic myeloid leukemia
Corresponding ICD-10 Codes
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)
Signs and Symptoms
Abnormal white blood count
Anemia
Bleeding complications/thrombotic
Fatigue
Fever
Night sweats
Progressive leukocytosis
Splenomegaly
Thrombocytopenia
Thrombocytosis
Weight loss
Progression and Transformation
Progression to the acute and blast phases
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
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: Myeloproliferative neoplasms
Pages: 30-36
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Myeloproliferative neoplasms
Pages: 30-36
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 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
National Cancer Institute
Section: General Information About Myeloproliferative Neoplasms
Pages: https://www.cancer.gov/types/myeloproliferative/hp/mds-mpd-treatment-pdq
Section: General Information About Myeloproliferative Neoplasms
Pages: https://www.cancer.gov/types/myeloproliferative/hp/mds-mpd-treatment-pdq
