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
Primary site must be bone marrow (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, (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, BCR-ABL1+
CML, Blast Phase (BP)
CML, Chronic Phase (CP)
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.
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.
Definitive Diagnostic Methods
Bone marrow biopsy
FISH
Genetic testing
Polymerase chain reaction (PCR)
Genetics Data
BCR-ABL1
Ph chromosome [del(22q)]
t(9;22)(q34;q11.2)
Immunophenotyping
None
Treatments
Blood thinners; anti-clotting medications; sometimes aspirin
Chemotherapy
Donor lymphocyte infusion (DLI)
Immunotherapy
Stem-cell transplant
Surgery (splenectomy)
Transformations from
None
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
Abnormal white blood count
Anemia
Bleeding complications/thrombotic
Destructive bone lesions/bone pain
Fatigue
Fever
Night sweats
Progressive leukocytosis
Splenomegaly
Thrombocytosis
Weight loss
Diagnostic Exams
Bone marrow aspiration and biopsy
Complete blood count (CBC)
Cytogenetic analsysis
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