Name

Classic Hodgkin lymphoma, lymphocyte-rich (LR-cHL)

ICD-O-3 Morphology

Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C770-C779
Lymph nodes are the most common primary sites.
Common metastatic sites include the bone, CNS, liver, lung, and bone marrow.
Spleen involvement is common due to dissemination of disease

Assign C779 if specific primary site cannot be determined.

See abstractor notes

Abstractor Notes

Classic Hodgkin lymphoma, lymphocyte-rich (LR-cHL) 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)

Lymphocyte-rich classical Hodgkin lymphoma (LRCHL) typically involves peripheral lymph nodes. Mediastinal involvement and bulky disease are uncommon. Most patients present with stage I or II disease. B symptoms are rare.

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

Hodgkin disease, lymphocyte predominance, NOS [OBS]
Hodgkin disease, lymphocyte-predominance, diffuse [OBS]
Hodgkin disease, lymphocytic-histiocytic predominance [OBS]
Hodgkin lymphoma, lymphocyte-rich

Definition

Classic Hodgkin lymphoma (CHL) is a neoplasm derived from germinal-centre B cells, characterized by a low fraction of tumour cells embedded in a reactive microenvironment rich in immune cells. The large neoplastic Hodgkin and Reed–Sternberg cells show a defective B-cell expression programme. (WHO 5th edition)

LRCHL, a subtype of CHL, is characterized by a nodular or, less commonly, diffuse infiltrate with abundant small lymphocytes, and it virtually lacks neutrophils and eosinophils.

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

Reed-Sternberg cells with clonal Ig gene rearrangements

Immunophenotyping

BOB1+ (expression/positive)
CD15+ (expression/positive)
CD20- (no expression/weak)
CD30+ (expression/positive)
CD45- (no expression/negative)
CD75- (no expression/negative)
OCT2- (decreased expression)
PAX5+ (expression/positive)

Treatments

Chemotherapy
Hormone therapy
Radiation therapy

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

C81.7 Other Hodgkin disease

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

C81.4_ Lymphocyte-rich Hodgkin lymphoma (effective October 01, 2015)
C81.4A Lymphocyte-rich Hodgkin lymphoma, 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)
Painless swelling in the lymph nodes
Peripheral 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
Erythrocyte sedimentation rate
Immunophenotyping
Lymph node biopsy
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Age: 30-50 years age group
Incidence: 5% of classical Hodgkin lymphoma
Sex: male predominance

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: Hodgkin lymphomas
Pages: Part B: 580-588

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 Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <02/12/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/adult-hodgkin-treatment-pdq. Accessed <03/30/2025>. [PMID: 26389473]
Section: Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-hodgkin-treatment-pdq
Glossary