Search Database ICD-O-3 Code Lists

Name

B-lymphoblastic leukemia/lymphoma [B-ALL/LBL] with hypodiploidy (Hypodiploid B-ALL/LBL)

ICD-O-3 Morphology

9816/3: B Lymphoblastic leukemia/lymphoma with hypodiploidy (Hypodiploid ALL)
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Module 4: Rules PH7, PH8 and Module 7 (for primary site for lymphoma presentation)
Usually presents as a leukemia (primary site C421). Lymphomatous presentation is rare.

See abstractor notes.

Coding Manual: Hematopoietic Coding Manual (PDF)

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].)

B-lymphoblastic leukemia/lymphoma [B-ALL/LBL] with hypodiploidy (Hypodiploid B-ALL/LBL) is part of the Precursor B-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B14)

All subtypes of ALL are coded as the same primary. If you have an ALL diagnosed, then a subsequent ALL diagnosed at a later time, this is the same primary.

If there are multiple diagnoses for ALL
- 9812/3-9819/3 take priority over 9811/3
- 9812/3 takes priority over 9813/3-9819/3
- Remaining combinations: Use the first diagnosis

B-ALL always involves the bone marrow and usually also involves the peripheral blood. Extramedullary involvement is common and is by definition the primary site of involvement in B-LBL, with frequent sites being the CNS, testes, lymph nodes, liver, spleen, skin, and soft tissues

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 hypodiploidy.

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.

Associated with Li-Fraumeni syndrome.

Diagnostic Confirmation

This histology can only be determined by positive genetics, diagnostic confirmation will always be 3.

Module Rule

Module 4: PH7, PH8

Alternate Names

B lymphoblastic leukemia/lymphoma, near-haploidy (B-ALL/LBL with near-haploidy)
B lymphoblastic leukemia/lymphoma, low hypodiploid (B-ALL/LBL with low hypodiploidy)
B lymphoblastic leukemia/lymphoma, high-hypodiploid (B-ALL/LBL with high hypodiploidy)

Definition

B-lymphoblastic leukemia/lymphoma (B-ALL/LBL) with hypodiploidy is a neoplasm of lymphoblasts of B-cell lineage defined by the presence of ≤ 43 chromosomes. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing

Genetics Data

Alterations in receptor tyrosine kinase and RAS pathway signaling (Near-haploid B-ALL/LBL)
Focal deletions of a histone gene cluster at 6p22 (Near-haploid B-ALL/LBL)
IKZF2 deletions ((Low-haploid B-ALL/LBL)
IKZF3 deletions (Near-haploid B-ALL/LBL)
RBI mutation ((Low-haploid B-ALL/LBL)
TP53 alterations (Low-haploid B-ALL/LBL)

Immunophenotyping

Immunophenotyping not relevant, diagnosed via genetic analysis

Treatments

Chemotherapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C83.5 Non-Hodgkin 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 [LBL] (effective October 01, 2015)
C83.5A Lymphoblastic (diffuse) lymphoma [LBL] in remission (effective October 01, 2024)
C91.0 Acute lymphoblastic leukemia [ALL] (effective October 01, 2015 - September 30, 2024)
C91.00 Acute lymphoblastic leukemia [ALL] not having achieved remission (effective October 01, 2024)
C91.01 Acute lymphoblastic leukemia [ALL], in remission (effective October 01, 2024)
C91.02 Acute lymphoblastic leukemia [ALL], in relapse (effective October 01, 2024)

Signs and Symptoms

Anemia
Arthralgias
Bone pain
Hepatomegaly
Lymphoadenopathy
Neutropenia
Splenomegaly
Thrombocytopenia

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
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
Survival: poor prognosis

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: B-lymphoblastic leukemia/lymphoma
Pages: Part B: 338-339

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

PDQ® Adult Treatment Editorial Board. PDQ Acute Lymphoblastic Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <02/13/2025>. Available at: https://www.cancer.gov/types/leukemia/hp/adult-all-treatment-pdq. Accessed <02/28/2025>. [PMID: 26389171]
Section: Acute Lymphoblastic Leukemia Treatment (PDQ®)–Health Professional Version
Pages: Acute Lymphoblastic Leukemia Treatment (PDQ®) - NCI
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