Search Database ICD-O-3 Code Lists

Name

Hodgkin lymphoma, nodular lymphocyte predominant (ICD-O-4: 96590/3)

ICD-O-3 Morphology

9659/3: Hodgkin lymphoma, nodular lymphocyte predominance
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

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is part of the Hodgkin lymphoma neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B16)

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.

T-cell histiocyte rich large B-cell lymphoma-LIKE transformation means that it is like a transformation. This is a variant of NLPHL and not an actual transformation to another lymphoma. Even though NLPHL can transform to T-cell histiocyte rich large B-cell lymphoma, it is not the case here since the word "like" appears in the diagnosis.

Diagnostic Confirmation

Assign code 1 for histological confirmation WITH or WITHOUT positive immunophenotyping or genetics

Module Rule

None

Alternate Names

Hodgkin paragranuloma, nodular [OBS] (ICD-O-4: 96590/3)
Hodgkin paragranuloma, NOS [OBS] (ICD-O-4: 96590/3)
Nodular lymphocyte predominant B-cell lymphoma (ICD-O-4: 96590/3)
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) (ICD-O-4: 96590/3)
T-cell histiocyte rich large B-cell lymphoma-LIKE transformation (ICD-O-4: 96590/3)

Definition

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a germinal centre–derived B-cell neoplasm composed of scattered large neoplastic B cells with multilobated nuclei (lymphocyte-predominant [LP] cells) within nodules dominated by mantle zone B cells and follicular dendritic cells (FDCs). Variant histological growth patterns also occur, in which small B cells are few and/or nodules are infrequent. (WHO 5th edition)

The WHO Classification of Hematopoietic and Lymphoid Tissues demonstrates six different patterns to NLPHL, which are: A) 'classical' nodular, B) serpiginous/interconnected nodular, C) nodular with prominent extra-nodular LP cells, D) T-cell-rich nodular, E) diffuse with a T-cell-rich background, and F) diffuse, B-cell-rich pattern.

Definitive Diagnostic Methods

Histologic confirmation

Genetics Data

Epstein–Barr Virus–Encoded Small RNAs (EBERs)
Reed Sternberg cells

Immunophenotyping

CD20, CD30, CD3, BCL6, OCT2, PDI, CD21

Treatments

Chemotherapy
Hormone therapy
Radiation therapy
Surgery

Transformations to

Transformations from

Same Primaries

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

C81.0 Hodgkin lymphocytic predominance

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

C81.0_ Nodular lymphocyte predominant Hodgkin lymphoma (effective October 01, 2015)
C81.0A Nodular lymphocyte predominant Hodgkin lymphoma, in remission (effective October 01, 2024)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Mediastinal mass
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Peripheral lymphadenopathy
Skin rash or itchy skin
Splenomegaly
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

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
Incidence: 5% of Hodgkin lymphomas
Sex: male predominance
Survival: 80% 10 year survival (stages I and II)

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: 591-598

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