Search Database ICD-O-3 Code Lists

Name

Lymphocyte-rich classic Hodgkin lymphoma

ICD-O-1 Morphology

9651/3: Hodgkin lymphoma, lymphocyte-predominance
Effective 1978 - 1991

ICD-O-2 Morphology

9657/3: Hodgkin disease, lymphocytic predominance, NOS
9658/3: Hodgkin disease, lymphocytic predominance, diffuse
Effective 1992 - 2000

ICD-O-3 Morphology

9651/3: Hodgkin lymphoma, lymphocyte-rich
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C770-C779
Lymph nodes (C770-C779) are the usual primary sites; however, involvement in other sites is possible. If you have confirmation that the only involved site is something other than the lymph nodes, then code to that primary site. See also Module 7.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

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.

Clinical features are similar to those of NLPHL with the exception that multiple relapses seem to occur less frequently. With modern risk-adjusted treatment, survival and relapse free survival are slightly better than the other subtypes of CHL.

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.

Grade

Not Applicable

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
LR-cHL
LRCHL

Definition

Lymphocyte-rich classic Hodgkin lymphoma (LRCHL) is a subtype of classic Hodgkin lymphoma (CHL) characterized by scattered Hodgkin/Reed-Sternberg (HRS) cells and a nodular or (less often) diffuse cellular background consisting of small lymphocytes, with an absence of neutrophils and eosinophils.

Definitive Diagnostic Methods

Histologic confirmation
Immunophenotyping

Genetics Data

None

Immunophenotyping

BCL6+ (expression/positive)
BOB1+ (expression/positive)
CD15+/- (positive/negative)
CD20+/- (positive/negative)
CD30+ (expression/positive)
CD57+/- (positive/negative)
CD75- (no expression/negative)
EBV/LMP1+ (expression/positive)
J chain- (no expression/negative)
IRF4/MUM1+ (expression/positive)
OCT2+ (expression/positive)
PAX5+/- (positive/negative)
PU1- (no expression/negative)
T follicular helper (TFH) cell immunophenotype (PD1/CDC279+)

Treatments

Chemotherapy
Hormone therapy
Radiation therapy

Transformations to

Transformations from

Same Primaries

Corresponding ICD-9 Codes

201.4 Hodgkin's disease, lymphocytic-histiocytic predominance

Corresponding ICD-10 Codes

C81.7 Other Hodgkin disease

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

C81.4 Lymphocyte-rich Hodgkin lymphoma (effective October 01, 2015)

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

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Hodgkin lymphomas
Pages: 438-440

International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 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

National Cancer Institute
Section: General Information About Adult Hodgkin Lymphoma (HL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-hodgkin-treatment-pdq
Glossary