Name

ICD-O-1 Morphology

9651/3: Hodgkin lymphoma, lymphocyte-predominance

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

Effective 2001 and later

Reportable

for cases diagnosed 1992 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.

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]
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.

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.

Definitive Diagnostic Methods

Histologic confirmation
Immunophenotyping

Genetics Data

None

Immunophenotyping

BCL6 expression
BOB1 expression
CD15 positive/negative
CD20 positive/negative
CD30 positive
CD57 positive/negative
CD75 negative
EBV/LMP1 positive
J chain negative
IRF4/MUM1 positive
OCT2 expression
PAX5 positive/negative
PU1 negative
T follicular helper (TFH) cell immunophenotype (PD1/CDC279+)

Treatments

Chemotherapy
Hormone therapy
Radiation therapy

Corresponding ICD-9 Codes

201.4 Lymphocytic-histiocytic predominance

Corresponding ICD-10 Codes

C81.7 Other Hodgkin's disease

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

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

Signs and Symptoms

Drenching night sweats
Fever (for no known reason)
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

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, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

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