Name

B-lymphoblastic leukemia/lymphoma with t(5;14)(q31.1;q32.1); IGH/IL3

ICD-O-3 Morphology

9817/3: B Lymphoblastic leukemia/lymphoma with t(5;14)(q31;q32); IL3-IGH
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(5;14)(q31.1;q32.1)
B lymphoblastic leukemia/lymphoma with t(5;14)(q31;q32); IL3-IGH

Definition

B-lymphoblastic leukemia/lymphoma with t(5;14)(q31.1;q32.1) is a neoplasm of lymphoblasts committed to the B-cell lineage in which the blasts harbor a translocation between IL3 and an IGH gene, resulting in variable eosinophilia.

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].)

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping

Genetics Data

Functional rearrangememt between the IL3 gene on chromosome 5 and the IGH@ gene on chromosome 14
Overexpression of IL3 gene

Immunophenotyping

CD10+
CD19+

Treatments

Chemotherapy

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

Progression and Transformation

None

Epidemiology and Mortality

Age: occurs in children and adults
Incidence: <1% of ALL
Survival: too few cases to be certain, although thought to be similar to other cases of ALL

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: 206-207

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