ICD-O-1 Morphology

9591/3: Malignant lymphoma, non-Hodgkin, NOS
9610/3: Lymphosarcoma, NOS
9630/3: Malignant lymphoma, lymphocytic, poorly differentiated, 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

9591/3: Malignant lymphoma, non-Hodgkin, NOS
9592/3: Lymphosarcoma, NOS
9593/3: Reticulosarcoma, NOS
9595/3: Malignant lymphoma, diffuse, NOS
9686/3: Malignant lymphoma, small cell, noncleaved, diffuse
9694/3: Malignant lymphoma, lymphocytic, intermediate differentiation, nodular
Effective 1992 - 2000

ICD-O-3 Morphology

9591/3: Malignant lymphoma, non-Hodgkin, NOS
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Abstractor Notes and Module 7

Grade

Not Applicable

Module Rule

None

Alternate Names

Hairy cell leukemia-variant
HCL-v
Lymphomatosis cerebri
Lymphosarcoma, diffuse [OBS]
Lymphosarcoma, NOS [OBS]
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, NOS [OBS]
NHL
Pleomorphic variant of HCL
Prolymphocytic variant of hairy cell leukemia
Reticulosarcoma, diffuse [OBS]
Reticulosarcoma, NOS [OBS]
Reticulum cell sarcoma, diffuse [OBS]
Reticulum cell sarcoma, NOS [OBS]
SDRPL
SMZL diffuse variant
Splenic B-cell lymphoma/leukemia, unclassifiable
Splenic diffuse red pulp small B-cell lymphoma
Splenic lymphoma with villous lymphocytes
Splenic marginal zone diffuse variant
Splenic red pulp lymphoma with numerous basophilic villous lymphocytes
Thrombocytic variant of hairy-cell leukemia
Thrombocytic variant of HCL

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.

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.

Definitive Diagnostic Methods

Bone marrow biopsy
Clinical diagnosis
Histologic confirmation
Immunophenotyping

Genetics Data

None

Immunophenotyping

Annexin A1 negative
CD5 positive
CD10 negative
CD11c+ (HCL-v)
CD11c negative (splenic red pulp disease)
CD20 positive
CD23 negative
CD25 negative
CD103 (HCL-v) positive
CD103 negative
CD123 (splenic red pulp disease) negative
DBA.44 (CD72) positive
IgD negative
IgG 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

Corresponding ICD-10 Codes

C85.9 Non-Hodgkin's lymphoma, unspecified type

Corresponding ICD-10-CM Codes (U.S. only)

C85.9 Non-Hodgkin's lymphoma, unspecified (effective October 01, 2015)
C85.1 Unspecified B-cell lymphoma (effective October 01, 2015)
C83.9 Non-follicular (diffuse) lymphoma, unspecified (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
Fever (for no known reason)
Massive splenomegaly (hairy cell leukemia variant type)
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

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

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary