Lymphocyte-rich classical Hodgkin lymphoma

Name
Lymphocyte-rich classical Hodgkin lymphoma
ICD-O-2 Morphology
9657/3
Effective 1992 - 2000
ICD-O-3 Morphology
9651/3
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 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
Classical Hodgkin lymphoma, lymphocyte-rich
Hodgkin disease, lymphocyte predominance, NOS [OBS]
Hodgkin disease, lymphocyte-predominance, diffuse [OBS]
Hodgkin disease, lymphocytic-histiocytic predominance [OBS]
LR-cHL
LRCHL
Definition
Subtype of classical Hodgkin lymphoma characterized by background of abundant small lymphocytes. Reed-Sternberg cells have classic morphology and immunophenotype. Most common growth pattern nodular, diffuse pattern rarely seen. 40% associated with EBV.

Lymphocyte-rich classical Hodgkin lymphoma is a subtype of classical Hodgkin lymphoma with scattered Hodgkin and Reed-Sternberg cells with a nodular or less often diffuse cellular background consisting of small lymphocytes and an absence of neutrophils and eosinophils.
Abstractor Notes
Lymphocyte-rich classical Hodgkin lymphoma (LRCHL) typically involves peripheral lymph nodes. Mediastinal involvement and bulky disease are uncommon. Most patients present with stage I or II disease. B symptoms are rare.

Clinical features are similar to those of NLPHL with the exception that multiple relapses seem to occur less frequently. With modern risk-adjusted treatment, survival and progression free survival are slightly better than the other subtypes of CHL.

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) where the documentation does not specifically state B-cell.
Definitive Diagnostic Methods
Histologic confirmation
Immunophenotyping
Genetics Data
None
Immunophenotyping
CD15+/-
CD20+/-
CD30+
CD45-
CD75-
J chain-
Treatments
Chemotherapy
Hormone
Radiation
Transformations to
Transformation to DLBCL is rare
Corresponding ICD-9 Codes
201.4 Lymphocytic-histiocytic predominance
Corresponding ICD-10 Codes
C81.7 Other Hodgkin's disease
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C81.4 Lymphocyte-rich 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
CT (CAT) scan
Complete blood count (CBC)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Recurrence and Metastases
None
Epidemiology and Mortality
Age: 30-50 years age group
Incidence: 5% of classical Hodgkin lymphoma
Sex: male predominance
Survival: progression free survival