Search Database ICD-O-3 Code Lists

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.

Coding Manual: Hematopoietic Coding Manual (PDF)

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.

Diagnostic Confirmation

This histology can only be determined by positive genetics and/or immunophenotyping, diagnostic confirmation will always be 3.

Grade

Not Applicable

Module Rule

Module 4: PH7, PH8

Alternate Names

B-ALL with BCR-ABL1

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.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping
Peripheral blood smear

Genetics Data

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

Immunophenotyping

CD10+ (expression/positive)
CD19+ (expression/positive)
TdT+ (expression/positive)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

Corresponding ICD-9 Codes

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

Corresponding ICD-10 Codes

C83.5 Non-Hodgkin 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

Anemia
Arthralgias
Bone pain
Elevated white blood cell (WBC) count
Hepatomegaly
Lymphoadenopathy
Neutropenia
Splenomegaly
Thrombocytopenia

Diagnostic Exams

Blood chemistry studies
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
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, Second Revision. Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

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