Name

B-lymphoblastic leukemia/lymphoma with hyperdiploidy

ICD-O-3 Morphology

9815/3: B Lymphoblastic leukemia/lymphoma with hyperdiploidy
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

See abstractor notes

Alternate Names

Definition

B-lymphoblastic leukemia/lymphoma with hyperdiploidy is a neoplasm of lymphoblasts committed to the B-cell lineage whose blasts contain > 50 chromosomes (usually < 66), typically without translocations or other structural alterations.

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].)

The presenting features are generally similar to those of other patients with ALL. Cases of T-ALL should not be included in this code even though those patients have near tetraploid karyotypes.

Immunophenotyping will confirm that the patient has a B-lymphoblastic B-ALL; the genetic testing provides the information needed to classify this neoplasm as a B lymphoblastic leukemia/lymphoma with hyperdiploidy.

Definitive Diagnostic Methods

Bone marrow biopsy
FISH
Genetic testing
Immunophenotyping
Karyotyping

Genetics Data

Extra copies of chromosomes 21, X, 14 and 4

Immunophenotyping

CD10+
CD19+
CD34+
CD45 absent

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

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: 205-206

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013. (http://codes.iarc.fr/codegroup/2)
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