Chronic myelogenous leukemia, BCR-ABL1 positive

Name
Chronic myelogenous leukemia, BCR-ABL1 positive
ICD-O-2 Morphology
9863/3
Effective 1992 - 2000
ICD-O-3 Morphology
9875/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
C421

Help me code for diagnosis year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
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, t(9;22)(q34;q11), BCR-ABL positive [OBS]
Chronic myelogenous leukemia, Philadelphia chromosome, (Ph1) 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, BCR-ABL1+
CML, Accelerated phase (AP)
CML, Blast Phase (BP)
CML, Chronic Phase (CP)
Myelogenous leukemia, NOS
Definition
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.
CML has three phases: chronic, accelerated, and the blastic phase or blast crisis.
Abstractor Notes
85% of patients are diagnosed in the chronic, asymptomatic phase. The diagnoses is usually incidental 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. Confirming the presence of the Philadelphia chromosome is required for a definitive diagnosis of CML. The Philadelphia chromosome is identified in the bone marrow by FISH analysis. The 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 three phases: chronic, accelerated, and the blastic phase or blast crisis. The accelerated phase can last weeks to months. In the chronic phase the involvement is usually limited to blood, bone marrow and spleen, although the liver may be infiltrated. During the 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. The terminal phase is the 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.
Definitive Diagnostic Methods
Bone marrow biopsy
FISH analysis on bone marrow
Molecular blood test performed on blood or bone marrow to detect BCR-ABL1 gene
Genetics Data
Ph chromosome [del(22q)]
t(9;22)(q34;q11.2)
Immunophenotyping
None
Treatments
Blood thinners; anti-clotting medications; sometimes aspirin
Differs by phase (chronic, blast accelerated). See abstractor notes
Transformations from
No Transformations
Corresponding ICD-9 Codes
205.1 Chronic myeloid leukemia
Corresponding ICD-10 Codes
C92.1 Chronic myeloid leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C92.1 Chronic myeloid leukemia, BCR/ABL-positive
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None