Search Database ICD-O-3 Code Lists

Name

Malignant lymphoma, non-Hodgkin, NOS

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

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Malignant lymphoma, non-Hodgkin, NOS is part of the Mature B-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B15)

Non-Hodgkin lymphoma, NOS is a generic disease description. DCO cases or path report only cases may stay in this classification.

More specific non-Hodgkin lymphomas include the following histologies: 9596/3, 9597/3, 9671/3, 9763/3, 9678/3, 9679/3, 9680/3, 9687/3, 9688/3, 9689/3, 9690/3, 9691/3, 9695/3, 9698/3, 9699/3, 9700/3, 9701/3, 9702/3, 9705/3, 9708/3, 9709/3, 9712/3, 9714/3, 9715/3, 9716/3, 9717/3, 9718/3, 9719/3, 9724/3, 9725/3, 9726/3, 9735/3, 9737/3, 9738/3, 9823/3, 9827/3, 9837/3

In most cases, NOS histology is only the provisional 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. If no information is found in the medical record, follow-back to the attending physician should be done.

When a more specific diagnosis is identified, the histology should be changed to the more specific neoplasm name and code. See the Lineage Tables in the Hematopoietic manual for more information on NOS and more specific histologies.

There are two splenic lymphoma variants that are included with 9591/3
1. Splenic diffuse red pulp small B-cell lymphoma. In this neoplasm, the spleen, bone marrow, and peripheral blood are involved. The splenic hilar nodes are usually involved. Other lymph node involvement is rare. Splenomegaly is present. Primary site spleen (C422)

2. Splenic B-cell lymphoma/leukemia with prominent nucleoli. In this neoplasm, the spleen, bone marrow, and peripheral blood are involved. Peripheral lymphadenopathy is uncommon. Hepatomegaly may be present. Splenomegaly is present. Previously classified as HCL variant or CD5-negative B-prolymphocytic leukemia. Primary site spleen (C422)

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.

Module Rule

None

Alternate Names

B-cell lymphoma, NOS
Hairy cell leukemia variant (pleomorphic, prolymphocytic, thrombocytic)
Lymphosarcoma, diffuse [OBS]
Lymphosarcoma, 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, small cell, noncleaved, diffuse [OBS]
Malignant lymphoma, small cleaved cell, NOS [OBS]
Non-Hodgkin lymphoma (NHL), NOS
Reticulosarcoma, diffuse [OBS]
Reticulosarcoma, NOS [OBS]
Reticulum cell sarcoma, diffuse [OBS]
Reticulum cell sarcoma, NOS [OBS]
Splenic B-cell lymphoma/leukemia, unclassifiable
Splenic B-cell lymphoma/leukemia with prominent nuclei (SBLPN)
Splenic diffuse red pulp small B-cell lymphoma (SDRPL)

Definition

Non-Hodgkin lymphoma not further classified. This is not a commonly used pathology term.

9591/3 also includes
1. Splenic diffuse red pulp small B-cell lymphoma: a small B-cell lymphoma involving the spleen, bone marrow, and peripheral blood, characterized by diffuse infiltration of the splenic red pulp by a monomorphic lymphoid population associated with circulating tumor cells bearing cytoplasmic projections. (WHO 5th edition)

2. Splenic B-cell lymphoma/leukemia with prominent nucleoli (SBLPN): a splenic B-cell neoplasm with some but not all of the cytomorphological and immunophenotypic features of cells of hairy cell leukemia (HCL); it lacks BRAF mutation and is resistant to conventional HCL therapy. Characteristically, the cells have a single large nucleolus. (WHO 5th edition)

Definitive Diagnostic Methods

Clinical diagnosis
Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

8q24 complex karyotype
14q32 complex karyotype
BRAF mutation absent
Hypermutated genes IGHV (predominantly IGHV3-23, IGHV-34)
Trisomy 12

Immunophenotyping

ANXA1- (no expression/negative)
CD19+ (expression/positive)
CD20+ (expression/positive)
CD25- (no expression/negative)
CD79a+ (expression/positive)
Cyclin D1- (no expression/negative)
TRAP- (no expression/negative)

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-10 Codes (Cause of Death codes only)

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)
C85.1A Unspecified B-cell lymphoma, in remission (effective October 01, 2024)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Leukopenia
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
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

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Splenic B-cell lymphomas and leukemias
Pages: Part B: 389-393

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 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

PDQ® Adult Treatment Editorial Board. PDQ Indolent B-Cell Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <02/18/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/indolent-b-cell-lymphoma-treatment-pdq. Accessed <03/04/2025>. [PMID: 37437080]
Section: Indolent B-Cell Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary