Non-Hodgkin lymphoma, NOS

Name
Non-Hodgkin lymphoma, NOS
ICD-O-1 Morphology
9591/3
Effective 1978 - 1991
ICD-O-2 Morphology
9591/3
Effective 1992 - 2000
ICD-O-3 Morphology
9591/3
Effective 2001 and later
Reportable
for cases diagnosed 1978 and later
Primary Site(s)
See Abstractor Notes and Module 7

Help me code for diagnosis year :

Grade
6 - B-cell
Module Rule
None
Alternate Names
B-cell lymphoma, NOS
Hairy cell leukemia-variant/prolymphocytic variant of hairy cell leukemia
HCL-v
Lymphocytic lymphoma simulating hairy cell leukemia
Lymphomatosis cerebri
Lymphosarcoma, diffuse [OBS]
Lymphosarcoma, NOS [OBS]
Malignant lymphoma, cleaved cell, NOS [OBS]
Malignant lymphoma, diffuse, NOS
Malignant lymphoma, lymphocytic, intermediate differentiation, nodular [OBS]
Malignant lymphoma, lymphocytic, poorly differentiated, diffuse [OBS]
Malignant lymphoma, non-cleaved cell, NOS
Malignant lymphoma, non-Hodgkin, NOS
Malignant lymphoma, small cell, noncleaved, diffuse [OBS]
Malignant lymphoma, small cleaved cell diffuse [OBS]
Malignant lymphoma, small cleaved cell, NOS [OBS]
Malignant lymphoma, undifferentiated cell, non-Burkitt [OBS]
Malignant lymphoma, undifferentiated cell type, NOS [OBS]
NHL
Pleomorphic variant of hairy cell leukemia
Pleomorphic variant of HCL
Reticulosarcoma, diffuse [OBS]
Reticulosarcoma, NOS [OBS]
Reticulum cell sarcoma, diffuse [OBS]
Reticulum cell sarcoma, NOS [OBS]
SMZL diffuse variant
Splenic B-cell lymphoma/leukemia, unclassifiable
Splenic diffuse red pulp small B-cell lymphoma
Splenic marginal zone diffuse variant
Thrombocytic variant of hairy-cell leukemia
Thrombocytic variant of HCL
Definition
Non-Hodgkin lymphoma not further classified by cell type nor defined for lineage (B-cell, T-cell, etc.) This is not a commonly used pathology term.

See abstractor notes for definitions of splenic B-cell lymphoma/leukemia, unclassifiable and the two most common types.
Abstractor Notes
This code is defined as non-Hodgkin lymphoma, NOS in the ICD-O-3. If you are using the code for non-Hodgkin, NOS, the "NOS" histology is a generic disease description. DCO cases and path-report only cases may remain in this classification. Most commonly, however, a NOS histology is only the working diagnosis; the physician will run further diagnostic procedures and look for various clinical presentations to identify a more specific disease. Further review of the medical record should be done to look for the tests listed as definitive diagnosis. When a more specific diagnosis is identified, change the histology to the more definitive term and code. More specific diseases would be any of the specific non-Hodkin lymphoma histologies.

WHO assigns the non-Hodgkin lymphoma, NOS code to a number of neoplasms, one of which is splenic B-cell lymphoma/leukemia, unclassifiable, a term that describes a number of variably well-defined entities involving the spleen that do not fall into any of the other types of B-cell lymphoid neoplasms. When using this code to describe splenic B-cell lymphoma/leukemia do the necessary text documentation to differentiate this disease from the non-Hodgkin, NOS. The two best defined of these splenic B-cell variants are splenic diffuse red pulp small B-cell lymphoma and hairy cell leukemia-variant (all coded to 9591/3). All of these diagnoses are non-specific. When a more specific B-cell neoplasm is defined, the histology code should be changed to the more specific diagnosis. Splenic diffuse red pulp small B-cell lymphoma is a rare disorder. All cases are diagnosed at clinical stage IV with spleen, BM and PB involvement. Peripheral lymph node involvement is rare. There is diffuse involvement of the red pulp of the spleen.

Another neoplasm coded to the NHL, NOS code is thrombocytic variant of hairy cell leukemia/hairy cell leukemia variant (Do not confuse with hairy cell leukemia. This neoplasm must be described as a VARIANT.) HCL-v accounts for about 10% of hairy cell leukemia cases. Spleen, BM and PB are involved. Hepatomegaly and lymphadenopathy is uncommon. Involvement of other solid tissue is rare. For hairy cell leukemia variant, code primary site to C42.1 with histology code 9591/3.

This code is also used for lymphomatosis cerebri because there is no ICD-O-3 code for lymphomatosis cerebri. Lymphomatosis cerebri (LC) is a rare primary nervous system lymphoma (PCNSL). This disease presents as a diffuse, infiltrating process without the formation of a mass. Radiographs show diffuse white matter involvement without enhancement.
Definitive Diagnostic Methods
Bone marrow
Clinical diagnosis
DX method varies depending because this NOS term covers so many neooplasms
Histologic confirmation
Immunophenotyping
Genetics Data
None
Immunophenotyping
Annexin A1-
CD5-
CD10-
CD11c-
CD20+
CD23-
CD25-
CD103-
CD123-
DBA 44 (CD72)+
IgD-
IgG+
Treatments
Bone marrow transplant
Chemotherapy
Hormone
Radiation
Surgery
Transformations to
No Transformations
Transformations from
No Transformations
Same Primaries
Corresponding ICD-9 Codes
202.8 Other lymphoma
Corresponding ICD-10 Codes
C85.9 Non-Hodgkin's lymphoma, unspecified type
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C85.9 Non-Hodgkin's lymphoma, unspecified
C85.1 Unspecified B-cell lymphoma
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None