Search Database ICD-O-3 Code Lists

Name

ALK-positive large B-cell lymphoma (ALK+ LBCL)

ICD-O-3 Morphology

9737/3: ALK-positive large B-cell lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Module 7
Most common sites of involvement: lymph nodes, aerodigestive tract, and gastrointestinal tract.
Common metastatic sites: CNS, liver
Spleen involvement is common due to dissemination of disease

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 code 9684/3.)

ALK-positive large B-cell lymphoma (ALK+ LBCL) is part of the Mature B-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B15)

ALK+ LBCL arises in lymph nodes in roughly 75% of cases, with frequent involvement of cervical lymph nodes. A variety of extranodal sites have been reported, among them the upper aerodigestive tract, brain, gastrointestinal tract, liver, and spleen.

Patients often present with asymptomatic lymphadenopathy and B-symptoms.

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

None

Alternate Names

None

Definition

Anaplastic lymphoma kinase–positive large B-cell lymphoma (ALK+ LBCL) is a diffuse, monomorphic neoplasm of large B cells with a plasmablastic immunophenotype and ALK expression due to ALK rearrangement. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

ALK rearrangement
Gains or amplifications of MYC
Translocations, inversions, insertions involving ALK at chromosome 2p23
t(2;17)(p23;q23)

Immunophenotyping

Blimp1+ (expression/positive)
CD20- (no expression/negative)
CD138+ (expression/positive)
IRF4/MUMI+ (expression/positive)
VS38c+ (expression/positive)
XBP1+ (expression/positive)

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)

C83.3 Non-Hodgkin lymphoma large cell (diffuse)

Corresponding ICD-10-CM Codes (U.S. only)

C83.3_ Diffuse large B-cell lymphoma (effective October 01, 2015)
C83.3A Diffuse large B-cell lymphoma, in remission (effective October 01, 2024)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Mediastinal mass
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Weight loss (for no known reason)

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: 35-38 years median age (8-85 years range)
Incidence: <1% of DLBCL lymphomas
Sex: 3 (M) - 4.1 (F) ratio
Survival: <2 years

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: Large B-cell lymphomas
Pages: Part B: 481-483

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 Aggressive B-Cell Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <02/24/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/aggressive-b-cell-lymphoma-treatment-pdq. Accessed <03/19/2025>.
Section: Aggressive B-Cell Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/aggressive-b-cell-lymphoma-treatment-pdq
Glossary