Chronic myelogenous leukemia, BCR-ABL1 positive

Chronic myelogenous leukemia, BCR-ABL1 positive
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
for cases diagnosed 1992 and later
Primary Site(s)
Primary site must be bone marrow (C421)

9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
Alternate Names
Chronic granulocytic leukemia
Chronic granulocytic leukemia, BCR-ABL [OBS]
Chronic granulocytic leukemia, Philadelphia chromosome, (Ph1) positive [OBS]
Chronic granulocytic leukemia, t(9;22)(q34;q11) [OBS]
Chronic myelogenous leukemia, Philadelphia chromosome, (Ph1) positive [OBS]
Chronic myelogenous leukemia, Philadelphia chromosome, t(9;22)(q34;q11), BCR-ABL positive [OBS]
Chronic myelogenous leukemia, t(9;22)(q34;q11) [OBS]
Chronic myelogenous leukemia, t(9;22)(q34;q11.2) [OBS]
Chronic myeloid leukemia-Accelerated phase
Chronic myeloid leukemia-Blast phase
Chronic myeloid leukemia-Chronic phase
CML, Accelerated phase (AP)
CML, Blast Phase (BP)
CML, Chronic Phase (CP)
Myeloproliferative neoplasm that is consistently associated with the BCR-ABL1 fusion gene located in the Philadelphia chromosome; originates in an abnormal bone marrow stem cell.
Abstractor Notes
Diagnosis of this disease 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.

The bone marrow is examined by chromosome and/or molecular techniques for the Philadelphia chromosome, which is required for a definitive diagnosis of CML.

The Philadelphia chromosome is identified in the bone marrow by FISH analysis. Polymerase chain reaction (PCR) is a very specialized molecular blood test that can detect the BCR-ABL1 gene from a blood sample or bone marrow.

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 disease progression from the chronic phase. The disease; however, remains the same histology: Chronic myelogenous leukemia.
4. Terminal phase - last phase and survival is usually only weeks or months.

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.

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)
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Polymerase chain reaction (PCR)
Genetics Data
Ph chromosome [del(22q)]
Donor lymphocyte infusion (DLI)
Stem-cell transplant
Surgery (splenectomy)
Transformations from
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
Bleeding complications/thrombotic
Destructive bone lesions/bone pain
Night sweats
Progressive leukocytosis
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