Name

ICD-O-1 Morphology

9650/3: Hodgkin lymphoma, NOS
Effective 1978 - 1991

ICD-O-2 Morphology

9650/3: Hodgkin lymphoma, NOS
9661/3: Hodgkin granuloma
9662/3: Hodgkin sarcoma
Effective 1992 - 2000

ICD-O-3 Morphology

9650/3: Hodgkin lymphoma, NOS
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 others 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.

Abstractor Notes

9650/3 is used in ICD-O-3 to describe Hodgkin lymphoma, NOS and by WHO to describe classical Hodgkin lymphoma. Both terms are "NOS" or generic neoplasms descriptions. DCO cases or path report only cases stay in this category when no additional information is available.

Classical Hodgkin may be a final diagnosis, but Hodgkin lymphoma, NOS more commonly is the provisional diagnosis given at the start of the work-up process; further review of the medical record should be done. Look for additional tests such as immunophenotyping or genetics.

When a patient has a history of Hodgkin lymphoma, NOS or classical Hodgkin lymphoma and subsequently a more specific Hodgkin lymphoma is diagnosed, change the histology code to the more specific neoplasm/code.

More specific Hodgkin lymphomas include
9651-Lymphocyte-rich classical Hodgkin lymphoma
9652-Mixed cellularity classical Hodgkin lymphoma
9653-Lymphocyte-rich depleted classical Hodgkin lymphoma
9655-Hodgkin lymphoma, lymphocyte depletion, reticular
9659-Nodular lymphocyte predominant Hodgkin lymphoma

Classical Hodgkin lymphoma (CHL)
1. Cervical region lymph nodes involved (75%) followed by mediastinal, axillary, and paraaortic regions.
2. Non-axial lymph node groups such as mesenteric or epitrochlear LN are rarely involved.
3. Primary extranodal involvement is rare.
4. More than 60% have localized involvement.
5.Splenic involvement is not uncommon (20%) and is associated with an increased risk of extranodal dissemination.
6. Bone marrow involvement is less common (5%). BM infiltration indicates disseminated involvement (Stage IV).

Radiation and chemotherapy have made CHL curable in more than 85% of cases.

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

CHL
HL
Hodgkin disease, NOS
Hodgkin lymphoma, NOS
Malignant lymphoma, Hodgkin

Definition

Classic Hodgkin lymphoma is monoclonal lymphoid neoplasm composed of mononuclear Hodgkin cells and multinucleated Reed-Sternberg cells residing in infiltrate of non-neoplastic small lymphocytes, eosinophils, neutrophils, histiocytes, plasma cells, fibroblasts, collagen fibers.

Lymphoproliferative disorder defined histologically by characteristic Reed-Sternberg cells, characterized by paucity of malignant cells surrounded by abundant "bystander" cells.

Definitive Diagnostic Methods

FISH
Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

Reed-Sternberg cells with clonal Ig gene rearrangements

Immunophenotyping

CD14+ (expression/positive)
CD15+ (expression/positive)
CD30+ (expression/positive)
CD45- (no expression/negative)
CD75- (no expression/negative)

Treatments

Chemotherapy
Hormone therapy
Radiation therapy

Transformations to

None

Corresponding ICD-9 Codes

201.9 Hodgkin's disease, unspecified

Corresponding ICD-10 Codes

C81.9 Hodgkin disease, unspecified

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

C81.9 Hodgkin lymphoma, unspecified (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: bimodal peaks at 15 years and 35 years
Incidence: 95% of all Hodgkin lymphomas
Survival: curable in ~85% of cases

Sources

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