Nodular lymphocyte predominant Hodgkin lymphoma

Name
Nodular lymphocyte predominant Hodgkin lymphoma
ICD-O-2 Morphology
9659/3
Effective 1992 - 2000
ICD-O-3 Morphology
9659/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
C770-C779

Help me code for diagnosis year :

Grade
Code grade specified by pathologist. If no grade specified, code 9
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.
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. NLPHL represents approximately 5% of all Hodgkin lymphomas. Patients are predominantly male and most frequently in the 30-40 year age group. This disease develops slowly with fairly frequent relapses. It usually remains responsive to therapy and thus is rarely fatal. The prognosis of patients with stage I and stage II disease is very good with 10-year survival in more than 80% of cases. In some countries (France) stage I disease is not treated, especially in children, after resection of the affected lymph nodes.

Advanced stages have unfavorable prognosis. Progression to large B-cell lymphoma-like lesions have been reported.

Preferred primary sites are listed for this disease in the primary site field; however, involvement in other sites is possible, but rare. If you have confirmation that the only involved site is something other than the preferred primary listed, then code to that primary site.
Definitive Diagnostic Methods
Bone Marrow biopsy
Histologic confirmation
Immunophenotyping
Genetics Data
Clonally rearranged immunoglobulin (IG) genes
Immunophenotyping
BCL6+
CD30+
CD45+
CD75+
CD79a
EMA+
Treatments
Chemotherapy
Hormone
Radiation
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 (effective October 1, 2015 U.S. only)
C81.0 Nodular lymphocyte predominant Hodgkin lymphoma
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None