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.

Grade

Not Applicable

Module Rule

None

Alternate Names

CHL
Classic Hodgkin Lymphoma PTLD
HL
Hodgkin disease, NOS

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.

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.

Definitive Diagnostic Methods

FISH
Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

Reed-Sternberg cells with clonal Ig gene rearrangements

Immunophenotyping

CD14+
CD15+
CD30+
CD45-
CD75-

Treatments

Chemotherapy
Hormone therapy
Radiation therapy

Transformations to

None

Corresponding ICD-9 Codes

201.9 Hodgkin's disease, unspecified

Corresponding ICD-10 Codes

C81.7 Other Hodgkin's disease
C81.9 Hodgkin lymphoma, unspecified

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

C81.7 Other classical Hodgkin lymphoma (effective October 01, 2015)
C81.9 Hodgkin lymphoma, unspecified (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: bimodal peaks at 15 years and 35 years
Survival: curable in ~85% of cases

Sources

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