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

Acute lymphoblastic leukemia with favorable trisomies
B-ALL/LBL with hyperdiploidy

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

Complete blood count (CBC)
CT (CAT) scan
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: 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