Name

B-lymphoblastic leukemia/lymphoma with t(1;19)(q23;p13.3); TCF3-PBX1

ICD-O-3 Morphology

9818/3: B Lymphoblastic leukemia/lymphoma with t(1;19) (q23;p13.3);E2A-PBX1 (TCF3-PBX1
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-ALL/LBL with t(1;19)(q23;p13.3)
B lymphoblastic leukemia/lymphoma with t(1;19)(q23;p13.3); E2A-PBX1 (TCF3-PBX1)

Definition

B-lymphoblastic leukemia/lymphoma with t(1;19)(q23;p13.3) is a neoplasm of lymphoblasts committed to the B-cell lineage in which the blasts harbor a translocation between TCF3 (also known as E2A) on chromosome 19 and PBX1 on chromosome 1.

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].)

Hematologic Transplant and/or Endocrine Procedures treatment includes bone marrow transplant.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping

Genetics Data

HLF gene on chromosome 17 (rare)
TCF2-PBX1 translocation
TCF3 translocation, t(17;19) (rare)

Immunophenotyping

CD9+
CD10+ cytoplasmic heavy chain-positive pre-B-cell phenotype
CD19+
CD34-

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: more common in children, can occur in adults
Incidence: 6% of cases of B-ALL
Survival: poor prognosis, but changing due to more modern intensive therapy

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: 207

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