Name

B-lymphoblastic leukemia/lymphoma with hypodiploidy

ICD-O-3 Morphology

9816/3: B Lymphoblastic leukemia/lymphoma with hypodiploidy (Hypodiploid ALL)
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 hypodiploidy
B lymphoblastic leukemia/lymphoma with hypodiploidy (Hypodiploid ALL)
High hypodiploid ALL
Hypodiploid ALL
Low hypodiploid ALL
Near-diploid ALL
Near-haploid ALL

Definition

B-lymphoblastic leukemia/lymphoma with hypodiploidy is a neoplasm of lymphoblasts committed to the B-cell lineage whose blasts contain < 46 chromosomes. Hypodiploid ALL is divided into four subtypes
1) Near-haploid ALL (with 23-29 chromosomes)
2) Low hypodiploid ALL (with 33-39 chromosomes)
3) High hypodiploid ALL (with 40-43 chromosomes)
4) Near diploid ALL (with 44-45 chromosomes) (note: this definition is usually not included, at least for treatment purposes)

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].)

Hypodiploid ALL has a poor prognosis. The prognosis for this neoplasm depends on the number of chromosomes; those with 44-45 chromosomes have the best prognosis. Patients may fare poorly even if they do not have residual neoplasms following therapy.

Definitive Diagnostic Methods

Bone marrow biopsy
FISH
Flow cytometry
Genetic testing
Immunophenotyping
Karyotyping

Genetics Data

Loss of function of mutations TP53 and/or RB1 (Low hypodiploid ALL)
Loss of one or more chromosomes
RAS or receptor tyronise kinase mutations (Near-haploid ALL)

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

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: limited to childhood (although can be seen in adulthood)
Incidence: 5% of 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

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