Classical Hodgkin lymphoma

Name
ICD-O-1 Morphology
9650/3
Effective 1978 - 1991
ICD-O-2 Morphology
9650/3
Effective 1992 - 2000
ICD-O-3 Morphology
9650/3
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.

Help me code for diagnosis year :

Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
None
Alternate Names
CHL
HL
Hodgkin disease, NOS
Malignant lymphoma, Hodgkin
Definition
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 disease 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 disease.
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 disease (Stage IV).

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

Per WHO, Hodgkin lymphomas are primarily B-cell lymphomas. If the medical record states B-cell, code 6. Since there are Hodgkin lymphomas that are not B-cell, the default grade is 9 (unknown) when the documentation does not specifically state B-cell.
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
Radiation
Transformations to
None
Corresponding ICD-9 Codes
201.9 Hodgkin's disease, unspecified
Corresponding ICD-10 Codes
C81.9 Hodgkin's disease, unspecified
C81.7 Other Hodgkin's disease
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C81.9 Hodgkin lymphoma, unspecified
C81.7 Other classical Hodgkin lymphoma
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
Bone marrow aspiration and biopsy
CT (CAT) scan
Complete blood count (CBC)
MRI (magnetic resonance imaging)
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