Search Database ICD-O-3 Code Lists

Name

B-lymphoblastic leukemia/lymphoma [B-ALL/LBL], NOS

ICD-O-3 Morphology

9811/3: B lymphoblastic leukemia/lymphoma, NOS
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 [lymphomas] or 9836/3 [leukemia].)

B-lymphoblastic leukemia/lymphoma [B-ALL/LBL], NOS 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

In most cases, the NOS histology is only the provisional diagnosis; the physician will run further diagnostic procedures and look for various clinical presentations to identify a more specific disease. Further review of the medical record should be done to look for the tests listed as definitive diagnosis. If no information is found in the medical record, follow-back to the attending physician should be done.

More specific B-lymphoblastic leukemia/lymphomas are:
9812/3: B-(acute) lymphoblastic leukemia/lymphoma (B-ALL) with BCR::ABL1 fusion
9813/3: B-lymphoblastic leukemia/lymphoma with KMT2A rearrangement
9814/3: B-lymphoblastic leukemia/lymphoma with ETV6::RUNX1 fusion
9815/3: B-lymphoblastic leukemia/lymphoma with hyperdiploidy 
9816/3: B lymphoblastic leukemia/lymphoma with hypodiploidy (Hypodiploid ALL)
9817/3: B-lymphoblastic leukemia/lymphoma with IGH::IL3 fusion
9818/3: B-lymphoblastic leukemia/lymphoma with TCF3::PBX1 fusion
9819/3: B-lymphoblastic leukemia/lymphoma, BCR-ABL1-like

Starting in the WHO Blue Book, 5th edition for Hematolymphoid tumors, several new terms, based on genetics were added to 9811/3. These terms can be found below and in the alternate names. They will always be diagnostic confirmation code 3.

Specific Lymphoblastic leukemia/lymphomas coded as 9811/3 include (document in your text the specific leukemia):

9811/3: B-lymphoblastic leukemia/lymphoma with DUX4 rearrangement
9811/3: B-lymphoblastic leukemia/lymphoma, iAMP21
9811/3: B-lymphoblastic leukemia/lymphoma with MEF2D rearrangement
9811/3: B-lymphoblastic leukemia/lymphoma with MYC rearrangement
9811/3: B-lymphoblastic leukemia/lymphoma with NUTM1 rearrangement
9811/3: B-lymphoblastic leukemia/lymphoma with other defined genetic alteration
9811/3: B-lymphoblastic leukemia/lymphoma with PAX5 alteration
9811/3: B-lymphoblastic leukemia/lymphoma with PAX5 p.PB0R variant
9811/3: B-lymphoblastic leukemia/lymphoma with ZNF384 rearrangement

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

Module 4: PH7, PH8

Alternate Names

Acute lymphoblastic leukemia (ALL)
B-lymphoblastic leukemia/lymphoma with DUX4 rearrangement
B-lymphoblastic leukemia/lymphoma, iAMP21
B-lymphoblastic leukemia/lymphoma with MEF2D rearrangement
B-lymphoblastic leukemia/lymphoma with MYC rearrangement
B-lymphoblastic leukemia/lymphoma with NUTM1 rearrangement
B-lymphoblastic leukemia/lymphoma with other defined genetic alteration
B-lymphoblastic leukemia/lymphoma with PAX5 alteration
B-lymphoblastic leukemia/lymphoma with PAX5 p.PB0R variant
B-lymphoblastic leukemia/lymphoma with ZNF384 rearrangement
Common ALL (c-ALL) (see 9836/3 prior to 2010)
Common precursor B-ALL (see 9836/3 prior to 2010)
Lymphoblastoma [OBS] (see 9727/3 prior to 2010)
Malignant lymphoma, convoluted cell [OBS] (see 9727/3 prior to 2010)
Malignant lymphoma, lymphoblastic, NOS (see 9727/3 prior to 2010)
Pre-pre-B acute lymphoblastic leukemia (Pre-pre-B-ALL) (see 9836/3 prior to 2010)
Precursor B-cell lymphoblastic leukemia (Pre-B-ALL)
Precursor B-cell lymphoblastic lymphoma, NOS (Pre-B-ALL) (see 9728/3 prior to 2010)
Precursor cell lymphoblastic leukemia (see 9727/3 prior to 2010)
Pro-B acute lymphoblastic leukemia (Pro-B-ALL) (see 9836/3 prior to 2010)

Definition

B-lymphoblastic leukemia/lymphoma (B-ALL/LBL) is a neoplasm of precursor lymphoid cells committed to the B-cell lineage, involving bone marrow and usually peripheral blood (B-lymphoblastic leukemia [B-ALL]) and often involving nodal or extranodal sites, which by definition are the primary sites of involvement in B-lymphoblastic lymphoma (B-LBL). (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

DUX4 rearrangement
iAMP21
MEF2D rearrangement
MYC rearrangement
NUTMI rearrangement
PAX5/PAX 5 PBOR
ZNF384 rearrangement

Immunophenotyping

CD10+ (expression/positive)
CD19+ (expression/positive)
CD22+ (expression/positive)
CD24+ (expression/positive)
CD79a+ (expression/positive)
PAX5+ (expression/positive)
TdT+ (expression/positive)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Radiation therapy

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)
C91.01Acute 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
Elevated white blood cell (WBC) count
Hepatomegaly
Lymphadenopathy
Neutropenia
Splenomegaly
Thrombocytopenia

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunhistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biposy
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Age: primarily disease of children (75% of cases under 6 years age)
Incidence: 1-4.74/100,000 per year worldwide, 10% of lymphoblastic lymphomas
Sex. slight male predominance
Survival: ~80% of children cured; less than 50% of adults cured

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: 330-336, 340-341, 360-364

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: https://www.cancer.gov/types/leukemia/hp/adult-all-treatment-pdq
Glossary