Name

B-lymphoblastic leukemia/lymphoma with t(9;22)(q34.1;q11.2); BCR-ABL1

ICD-O-3 Morphology

9812/3: B Lymphoblastic leukemia/lymphoma with t(9;22)(q34;q11.2); BCR-ABL1
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.

Grade

Not Applicable

Module Rule

Module 4: PH7, PH8

Alternate Names

B-ALL with BCR-ABL11

Definition

B-lymphoblastic leukemia/lymphoma (B-ALL/LBL) with t(9;22)(q34.1;q11.2); BCR-ABL1 is a neoplasm of lymphoblasts committed to the B-cell lineage in which the blasts harbor a translocation between BCR on chromosome 22 and the ABL1 oncogene on chromosome 9.

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 neoplasm 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

ABL1 at 9q34.1
Fusion of BCR at 22q11.2
p190 kd BCR-ABL1 fusion protein

Immunophenotyping

CD10+
CD19+
TdT+

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

200.1 Lymphosarcoma (Lymphoma presentation)
204.0 Acute lymphoid leukemia (Leukemia presentation)

Corresponding ICD-10 Codes

C83.5 Diffuse non-Hodgkin's lymphoma, lymphoblastic (diffuse) (Lymphoma presentation)
C91.0 Acute lymphoblastic leukemia (Leukemia presentation)

Corresponding ICD-10-CM Codes (U.S. only)

C83.5 Lymphoblastic (diffuse) lymphoma (Lymphoma presentation) (effective October 01, 2015)
C91.0 Acute lymphoblastic leukemia [ALL] (Leukemia presentation) (effective October 01, 2015)

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

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: Precursor lymphoid neoplasms
Pages: 203

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Acute Lymphoid Leukemia
Pages: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq
Glossary