Name

Nodular lymphocyte predominant Hodgkin lymphoma

ICD-O-1 Morphology

9660/3: Hodgkin paragranuloma

ICD-O-2 Morphology

9659/3: Hodgkin lymphoma, nodular lymphocyte predominance
9660/3: Hodgkin paragranuloma
Effective 1992 - 2000

ICD-O-3 Morphology

9659/3: Hodgkin lymphoma, nodular lymphocyte predominance
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 Module 7

Grade

Not Applicable

Module Rule

None

Alternate Names

Hodgkin lymphoma, lymphocyte predominance, nodular
Hodgkin lymphoma, nodular lymphocyte predominance
Hodgkin paragranuloma, nodular [OBS]
Hodgkin paragranuloma, NOS [OBS]
NLPHL

Definition

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a B-cell neoplasm usually characterized by a nodular or a nodular and diffuse proliferation of small lymphocytes with single scattered large neoplastic cells known as lymphocyte predominant (LP) or popcorn cells, formerly called L&H cells for lymphocytic and/or histiocytic Reed-Sternberg cell variants. The LP cells are ringed by PD1/CD279+ T-cells in almost all instances. In typical cases, the LP cells reside in large nodular meshworks of follicular dendritic cell (FDC) processes that are filled non-neoplastic lymphocytes (mainly B cells) and histiocytes.

Abstractor Notes

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) commonly involves cervical, axillary, or inguinal lymph nodes. Mediastinal, splenic, and bone marrow involvement are rare.

Most patients present with localized peripheral lymphadenopathy (stage I or II). 5-25% of patients present with advanced stage disease.

The disease is usually responsive to therapy and thus is rarely fatal.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

Clonally rearranged immunoglobulin IG IGHV) genes
EBV encoded small RNA (EBER)

Immunophenotyping

BCL6 positive
BOB1 positive
CD10 absent
CD15 lacking
CD20 positive
CD30 lacking
CD45 positive
CD75 positive
CD79a positive
EMA positive
IgD positive
IgM negative
OCT2 positive
PAX5 positive

Treatments

Chemotherapy
Hormone therapy
Radiation therapy
Surgery

Corresponding ICD-9 Codes

201.4 Hodgkin's disease, lymphocytic-histiocytic predominance

Corresponding ICD-10 Codes

C81.0 Hodgkin's disease, lymphocytic predominance

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

C81.0 Nodular lymphocyte predominant Hodgkin lymphoma (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
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)

Progression and Transformation

Advanced stages have unfavorable prognosis
Disease develops slowly with frequent relapses
Transformation to diffuse large b-cell lymphoma is possible

Epidemiology and Mortality

Age: 30-50 years range most common
Sex: male predominance
Survival: 80% 10 year survival (stages I and II)

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: 431-434

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