Name

B-lymphoblastic leukemia/lymphoma with t(12;21) (p13.2;q22.1); ETV6-RUNX1

ICD-O-3 Morphology

9814/3: B Lymphoblastic leukemia/lymphoma with t(12;21)(p13;q22); TEL-AML1 (ETV6-RUNX1
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. Lymphomatous presentation is rare.

Grade

Not Applicable

Module Rule

Module 4: PH7, PH8

Alternate Names

B lymphoblastic leukemia/lymphoma with iAMP21
B lymphoblastic leukemia/lymphoma with t(12;21)(p13;q22); TEL-AML1 (ETV6-RUNX1)
B-ALL/LBL with t(12;21)(p13.2;q22.1)

Definition

B-lymphoblastic leukemia/lymphoma with t(12;21) (p13.2;q22.1) is a neoplasm of lymphoblasts committed to the B-cell lineage in which the blasts harbor a translocation between ETV6 (also called TEL) on chromosome 12 and RUNX1 (also called AML1) on chromosome 21.

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 leukemia is relatively common in children. The incidence decreases with age, so it is seldom seen in older children and rarely, if never, in adults.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping

Genetics Data

t(12;21)(p13;q22); ETV6-RUNX1 translocation

Immunophenotyping

CD9-
CD10+
CD13 expression
CD19+
CD20-
CD34+
CD66c-

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)

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: most common in children (not seen in infants, rare in adulthood)
Incidence: 25% of cases of B-ALL
Survival: favorable prognosis, >90% cure of children

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: 204-205

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