B lymphoblastic leukemia/lymphoma with t(9;22)(q34;q11.2); BCR-ABL1

Name
B lymphoblastic leukemia/lymphoma with t(9;22)(q34;q11.2); BCR-ABL1
ICD-O-3 Morphology
9812/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
See Module 4: Rules PH7, PH8
Usually presents as a leukemia. Organ involvement is possible. Lymphomatous presentation is rare.

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Grade
6 - B-cell
Module Rule
Module 4: PH7, PH8
Alternate Names
None
Definition
This neoplasm is committed to the B-cell lineage in which the blasts harbour a translocation between the BCR gene on chromosome 22 and the ABL1 oncogene on chromosome 9.

Patients with B-ALL may have organ involvement, but lymphomatous presentations are rare.
Abstractor Notes
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see codes: 9728/3 [lymphoma] or 9836/3 [leukemia].)

This disease occurs in both children and adults. Children usually have a better outcome.

This neoplasm is confirmed by immunological markers and/or ultrastructural cytochemistry.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Peripheral blood smear
Genetics Data
ABL-1 at 9q34
Fusion of BCR at 22q11.2
p190 kd BCR-ABL1 fusion protein
Immunophenotyping
CD10+
CD19+
TdT+
Treatments
Chemotherapy
Stem cell transplant
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
204.8 Other lymphoid leukemia
Corresponding ICD-10 Codes
C91.7 Other lymphoid leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C91.Z Other lymphoid leukemia
Signs and Symptoms
Diagnostic Exams
CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Progression and Transformation
None
Epidemiology and Mortality
Age: more common in adults than children
Incidence: 25% of adults ALL (2-% of childhood ALL)
Survival: worst prognosis of the ALL's