Nodular lymphocyte predominant Hodgkin lymphoma

Name
Nodular lymphocyte predominant Hodgkin lymphoma
ICD-O-2 Morphology
9659/3: Hodgkin lymphoma, nodular lymphocyte predominance
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

Help me code for diagnosis year :

Grade
6 - B-cell
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
Monoclonal B-cell neoplasm characterized by nodular or nodular and diffuse, polymorphous proliferation of scattered large neoplastic cells, "popcorn" or L&H (lymphocytic and/or histiocytic) cells. L&H cells reside in large spherical meshworks of follicular dendritic cell process filled with non-neoplastic lymphocytes and histiocytes.
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.

In some countries (France) stage I disease is not treated, especially in children, after resection of the affected lymph nodes.

For more information on this neoplasm, see the NCI website: http://www.cancer.gov/types/lymphoma/hp/adult-hodgkin-treatment-pdq or http://www.cancer.gov/types/lymphoma/patient/adult-hodgkin-treatment-pdq
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Clonally rearranged immunoglobulin (IG) genes
Immunophenotyping
BCL6+
CD20+
CD45+
CD75+
CD79a+
EMA+
Treatments
Chemotherapy
Hormone therapy
Radiation therapy
Surgery
Corresponding ICD-9 Codes
201.4 Hodgkin's disease, lymphocytic-histiocytic predominance
Corresponding ICD-10 Codes
C81.0 Hodgkin's disease, 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
Fever (for no known reason)
Mediastinal mass
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
Advanced stages have unfavorable prognosis
Disease develops slowly with frequent relapses
Transformation to diffuse large b-cell lymphoma is possible
Epidemiology and Mortality
Age: 30-50 years range most common
Sex: male predominance
Survival: 80% 10 year survival (stages I and II)