Search Database ICD-O-3 Code Lists

Name

ALK-positive anaplastic large cell lymphoma (ALCL)

ICD-O-3 Morphology

9714/3: Anaplastic large cell lymphoma, T cell and Null cell type
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

Module 7
Most common sites of involvement: lymph nodes, skin, soft tissue.
Common metastatic sites: bone, CNS, liver, lung, bone marrow

See abstractor notes.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

ALK-positive anaplastic large cell lymphoma (ALCL) is part of the Mature T-cell and NK-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B20)

Lymph nodes are commonly involved, and extranodal involvement is frequent, most commonly the skin, bone, soft tissue and lung.

Bone marrow involvement is present in about 15% of patients.

Patients usually present with advanced-stage disease.

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

ALK positive anaplastic large cell lymphoma, common
ALK positive anaplastic large cell lymphoma, composite
ALK positive anaplastic large cell lymphoma, Hodgkin like
ALK positive anaplastic large cell lymphoma, lymphohistiocytic
ALK positive anaplastic large cell lymphoma, small cell
Anaplastic large cell lymphoma, ALK-positive (ALK+ ALCL)
Anaplastic large cell lymphoma, CD30 positive
Anaplastic large cell lymphoma, T cell and Null cell type
Large cell (Ki-1 positive) lymphoma [OBS]

Definition

ALK-positive anaplastic large cell lymphoma (ALCL) is a CD30-positive mature T-cell lymphoma with aberrant expression of ALK secondary to rearrangements of the ALK gene. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

t (2;5) (p23;q35)
NPM1::ALK fusion protein

Immunophenotyping

Aberrant expression of ALK secondary to ALK rearrangement
CD2+ (expression/positive)
CD4+ (expression/positive)
CD25+ (expression/positive)
CD30+ (expression/positive)
CD43+ (expression/positive)
CD45+ (expression/positive)
CD45RO+ (expression/positive)
EMA+ (expression/positive)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy

Transformations to

None

Transformations from

Same Primaries

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

C84.6 Anaplastic large cell lymphoma, ALK-positive

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

C84.6_ Anaplastic large cell lymphoma, ALK-positive (effective October 01, 2015)
C84.6A Anaplastic large cell lymphoma, ALK-positive, in remission (effective October 01, 2024)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Peripheral and abdominal lymphadenopathy
Skin rash or itchy skin
Weight loss (for no known reason)

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
Peripheral blood smear
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Incidence: 10-15% of pediatric and adolescent NHL's/3% of adult NHL's
Age: median age 30 years
Sex: M:F ratio 3.1
Survival: 5-year survival ~80%

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: Mature T- and NK-cell neoplasms
Pages: Part B: 731-733

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 Peripheral T-Cell Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <03/13/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/peripheral-t-cell-lymphoma-pdq. Accessed <03/31/2025>. [PMID: 37437079]
Section: Peripheral T-Cell Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/peripheral-t-cell-lymphoma-pdq#_2289
Glossary