Name
Nodular lymphocyte predominant Hodgkin lymphoma
ICD-O-1 Morphology
9660/3: Hodgkin paragranuloma
ICD-O-2 Morphology
9659/3: Hodgkin lymphoma, nodular lymphocyte predominance
9660/3: Hodgkin paragranuloma
Effective
1992 - 2000
ICD-O-3 Morphology
9659/3: Hodgkin lymphoma, nodular lymphocyte predominance
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 Module 7
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
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.
The disease is usually responsive to therapy and thus is rarely fatal.
Most patients present with localized peripheral lymphadenopathy (stage I or II). 5-25% of patients present with advanced stage disease.
The disease is usually responsive to therapy and thus is rarely fatal.
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
Hodgkin lymphoma, lymphocyte predominance, nodular
Hodgkin lymphoma, nodular lymphocyte predominance
Hodgkin paragranuloma, nodular [OBS]
Hodgkin paragranuloma, NOS [OBS]
NLPHL
Definition
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a B-cell neoplasm usually characterized by a nodular or a nodular and diffuse proliferation of small lymphocytes with single scattered large neoplastic cells known as lymphocyte predominant (LP) or popcorn cells, formerly called L&H cells for lymphocytic and/or histiocytic Reed-Sternberg cell variants. The LP cells are ringed by PD1/CD279+ T-cells in almost all instances. In typical cases, the LP cells reside in large nodular meshworks of follicular dendritic cell (FDC) processes that are filled non-neoplastic lymphocytes (mainly B cells) and histiocytes.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Clonally rearranged immunoglobulin IG IGHV) genes
Immunophenotyping
BCL6+ (expression/positive)
BOB1+ (expression/positive)
CD10- (no expression/negative)
CD15- (no expression/lacking/negative)
CD20+ (expression/positive)
CD30- (no expression/lacking/negative)
CD45+ (expression/positive)
CD75+ (expression/positive)
CD79a+ (expression/positive)
EMA+ (expression/positive)
IgD+ (expression/positive)
IgM- (no expression/negative)
OCT2+ (expression/positive)
PAX5+ (expression/positive)
Treatments
Chemotherapy
Hormone therapy
Radiation therapy
Surgery
Transformations to
Transformations from
Same Primaries
Corresponding ICD-9 Codes
201.4 Hodgkin's disease, lymphocytic-histiocytic predominance
201.0 Hodgkin's paragranuloma
Corresponding ICD-10 Codes
C81.0 Hodgkin lymphocytic predominance
Corresponding ICD-10-CM Codes (U.S. only)
C81.0 Nodular lymphocyte predominant Hodgkin lymphoma (effective October 01, 2015)
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
Epidemiology and Mortality
Sources
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Hodgkin lymphomas
Pages: 431-434
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Hodgkin lymphomas
Pages: 431-434
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
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
Section: General Information About Adult Hodgkin Lymphoma (HL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-hodgkin-treatment-pdq