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Name
Non-Hodgkin lymphoma, NOS
ICD-O-1 Morphology
9610/3: Lymphosarcoma, NOS
9631/3: Prolymphocytic lymphosarcoma
9640/3: Reticulosarcoma, NOS
9641/3: Reticulosarcoma, pleomorphic cell type
9642/3: Reticulosarcoma, nodular
Effective
1978 - 1991
ICD-O-2 Morphology
9592/3: Lymphosarcoma, NOS
9593/3: Reticulosarcoma, NOS
Effective
1992 - 2000
Reportable
for cases diagnosed
1978 and later
Primary Site(s)
See Abstractor Notes and Module 7
Coding Manual:
Hematopoietic Coding Manual (PDF)
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-Hodgkin lymphoma histologies.
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-Hodgkin lymphoma histologies.
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
B-cell lymphoma, NOS
Hairy cell leukemia-variant (thrombocytic) (HCL-v)
Lymphocytic lymphoma simulating hairy cell leukemia
Lymphomatosis cerebri
Lymphosarcoma, NOS [OBS]
Malignant lymphoma, non-Hodgkin, NOS
Pleomorphic variant of hairy cell leukemia
Pleomorphic variant of HCL
Prolymphocytic variant of hairy cell leukemia
Reticulosarcoma, diffuse [OBS]
Reticulosarcoma, NOS [OBS]
Definition
Non-Hodgkin lymphoma not further classified. This is not a commonly used pathology term.
There are two main variants for NHL, NOS. Tese are assigned histology code 9591/3.
1) Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is an uncommon lymphoma with a diffuse pattern of involvement of the splenic red pulp by small monomorphous B lymphocytes. The neoplasm also involves bone marrow sinusoids and peripheral blood.
2) Hairy cell leukemia variant (HCL-v) encompasses cases of B-cell chronic lymphoproliferative disorders that resemble classic HCL but exhibit variant cytological and hematological features such as leukocytosis, presence of monocytes, cells with prominent nucleoli, cells with blastic or convoluted nuclei, and/or absence of circumferential shaggy contours. It is also called prolymphocytic variant of hairy cell leukemia.
There are two main variants for NHL, NOS. Tese are assigned histology code 9591/3.
1) Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is an uncommon lymphoma with a diffuse pattern of involvement of the splenic red pulp by small monomorphous B lymphocytes. The neoplasm also involves bone marrow sinusoids and peripheral blood.
2) Hairy cell leukemia variant (HCL-v) encompasses cases of B-cell chronic lymphoproliferative disorders that resemble classic HCL but exhibit variant cytological and hematological features such as leukocytosis, presence of monocytes, cells with prominent nucleoli, cells with blastic or convoluted nuclei, and/or absence of circumferential shaggy contours. It is also called prolymphocytic variant of hairy cell leukemia.
Definitive Diagnostic Methods
Bone marrow biopsy
Clinical diagnosis
Histologic confirmation
Immunophenotyping
Genetics Data
None
Immunophenotyping
Annexin A1- (no expression/negative)
CD5+ (expression/positive)
CD10- (no expression/negative)
CD11c (HCL-v)+ (expression/positive)
CD11c (splenic red pulp disease)- (no expression/negative)
CD20+ (expression/positive)
CD23- (no expression/negative)
CD25- (no expression/negative)
CD103 (HCL-v)+ (expression/positive)
CD103- (no expression/negative)
CD123 (splenic red pulp disease)- (no expression/negative)
DBA.44 (CD72)+ (expression/positive)
IgD- (no expression/negative)
IgG+ (expression/positive)
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Radiation therapy
Surgery
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Same Primaries
Corresponding ICD-9 Codes
202.8 Other lymphoma
200.0 Reticulosarcoma
Corresponding ICD-10 Codes
C85.1 B-cell lymphoma, unspecified
C85.0 Lymphosarcoma
Corresponding ICD-10-CM Codes (U.S. only)
C85.1 Unspecified B-cell lymphoma (effective October 01, 2015)
Signs and Symptoms
Drenching night sweats
Fatigue
Fever (for no known reason)
Leukopenia
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Thrombocytopenia
Weight loss (for no known reason)
Diagnostic Exams
Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
Peripheral blood smear
PET (positron emission tomography) scan
Progression and Transformation
None
Epidemiology and Mortality
None
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: Mature B-cell neoplasms
Pages: 229-231
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 229-231
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 Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
