Search Database ICD-O-3 Code Lists

Name

Diffuse large B-cell lymphoma, NOS (DLBCL)

ICD-O-3 Morphology

9680/3: Malignant lymphoma, large B-cell, diffuse, NOS
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Abstractor Notes and Modules 6 and 7
DLBLC can occur anywhere in the body.
Common metastatic sites include bone, CNS, liver, lung, and bone marrow.

Spleen involvement is common due to dissemination of disease. In rare cases, DLBCL may occur de novo in the spleen (no other involvement found)

See abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Diffuse large B-cell lymphoma (DLBCL), 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)

Virtually any site in the body can be involved with DLBCL. Lymph nodes are the most common. Common extranodal sites include the GI tract, head and neck, CNS, bone, liver, kidney and adrenal gland.

Unlike other lymphomas, DLBCL can originate in the spleen. Only assign spleen as the primary site when the spleen is the only thing involved.

DLBCL can occur de novo or be the result of a transformation from an indolent lymphoma (i.e., CLL/SLL, Follicular lymphoma). See Rules M8-M13 if there is a transformation involving DLBCL.
-Richter syndrome/transformation is when CLL/SLL (9823/3) transforms to DLBCL.

There are several subtypes of diffuse large B-cell lymphoma
1. ALK-positive large B-cell lymphoma (9737/3)
2. Diffuse large B-cell lymphoma associated with chronic inflammation (9680/3)
3. Diffuse large B-cell lymphoma with MYC and BLC2 rearrangements (9680/3)
4. DLBCL/HGBCL-MYC/BCL2 with BCL6 rearrangement
5. DLBCL/HGBCL-MYC/BCL2 without BCL6 rearrangement
6. DLBCL/HGBCL-MYC/BCL2 (with or without BCL6
rearrangement (with TdT expression)
7. EBV-positive diffuse large B-cell lymphoma, NOS (9680/3)
8. Fibrin-associated large B-cell lymphoma (9678/3)
9. Fluid overload-associated large B-cell lymphoma (9678/3)
10. High-grade B-cell lymphoma, NOS (9680/3)
11. High-grade B-cell lymphoma with MYC and BCL2 rearrangements (9680/3)
12. High-grade B-cell lymphoma with 11q aberration (9687/3)
13. Intravascular large B-cell lymphoma (9712/3)
14. Large B-cell lymphoma with IRF4 rearrangement (9698/3)
15. Lymphomatoid granulomatosis (9766/3)
16. Mediastinal grey zone lymphoma (9596/3)
17. Plasmablastic lymphoma (9735/3)
18. Primary large B-cell lymphoma of immune-privileged sites (9680/3)
19. Primary mediastinal large B-cell lymphoma (9679/3)
20. T-cell/histiocyte-rich large B-cell lymphoma (9688/3)

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

Module 6: PH11 (primary sites C44_)

Alternate Names

Anaplastic large B-cell lymphoma (ALK status unknown)
B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma (DLBCL/BL)
Diffuse large B-cell lymphoma, activated (non-germinal centre) B-cell subtype
Diffuse large B-cell lymphoma, anaplastic subtype
Diffuse large B-cell lymphoma, centroblastic subtype
Diffuse large B-cell lymphoma, germinal centre B-cell subtype
Diffuse large B-cell lymphoma, immunoblastic subtype (see 9684/3 prior to 2010+)
Diffuse large B-cell lymphoma with MYC and BCL6 rearrangements
DLBCL/HGBCL-MYC/BCL with or without BCL6 rearrangements
DLBCL/HGBCL-MYC/BCL2 with or without BCL6 rearrangement (with TdT expression)
Double hit lymphoma
EBV positive diffuse large B-cell lymphoma (EVB-DLBCL)
Fibrin-associated large B-cell lymphoma (FA-LBCL) (see 9678/3 for 2026+)
Fluid overload associated B-cell lymphoma (FO-LBCL) (see 9678/3 for 2026+)
Fluid-overload effusion lymphoma (see 9678/3 for 2026+)
High grade B-cell lymphoma, NOS with MYC and BCL6 rearrangement
High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements
Immunodeficiency-associated lymphoproliferative disorders
Malignant lymphoma, centroblastic, diffuse [OBS]
Malignant lymphoma, centroblastic, NOS [OBS]
Malignant lymphoma, histiocytic, diffuse [OBS]
Malignant lymphoma, histiocytic, NOS [OBS]
Malignant lymphoma, large B-cell, diffuse, centroblastic, NOS {OBS]
Malignant lymphoma, large B-cell, diffuse, NOS
Malignant lymphoma, large B-cell, NOS
Malignant lymphoma, large cell, cleaved, NOS [OBS]
Malignant lymphoma, large cell, cleaved and non cleaved [OBS]
Malignant lymphoma, large cell, diffuse, NOS [OBS]
Malignant lymphoma, large cell, noncleaved, diffuse, NOS [OBS]
Malignant lymphoma, large cell, noncleaved, NOS [OBS]
Malignant lymphoma, large cell, NOS
Malignant lymphoma, large cleaved cell, NOS [OBS]
Malignant lymphoma, noncleaved, diffuse, NOS [OBS]
Malignant lymphoma, noncleaved, NOS
Primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL-LT) (C447)
Primary large B-cell lymphoma of immune-privileged sites (IP-LBCL)
Primary large B-cell lymphoma of the CNS (PCNS-LBCL) (C70_, C71_, C72_)
Primary large B-cell lymphoma of the Testis (PT-LBCL) (C62_)
Primary large B-cell lymphoma of the vitreoretinal (PVR-LBCL) (C69_) (excludes choroid)
Primary intraocular lymphoma (PIOL)
Primary vitreoretinal large B-cell lymphoma
Richter syndrome/transformation (CLL/SLL transforms to DLBCL)
Triple hit lymphoma

Definition

The family/class of large B-cell lymphomas comprises a spectrum of tumors with varying morphologies, genetic features, and clinical behaviors. Diffuse large B-cell lymphoma (DLBCL) NOS represents the largest entity and is defined by morphology and a mature B-cell phenotype. (WHO 5th edition)

Subgroups of DLBCL, NOS
1. Diffuse large B-cell lymphoma (DLBCL) NOS: A morphologically and molecularly heterogeneous entity that does not meet the diagnostic criteria for specific large B-cell lymphoma neoplasms.

2. Diffuse large B-cell lymphoma / high-grade B-cell lymphoma with MYC and BCL2 rearrangements (DLBCL/HGBCL-MYC/BCL2): Aggressive mature B-cell lymphoma with structural chromosomal aberrations with breakpoints at both MYC and BCL2 loci.

3. Epstein Barr virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL): A large B-cell lymphoma in which the majority of the neoplastic cells harbor EBV.

4. Primary large B-cell lymphomas (LBCLs) of immune-privileged sites (IP-LBCLs) comprise LBCLs that arise as primary tumours in the CNS, vitreoretina, and testis of immunocompetent patients.

5. Primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL-LT): Most commonly arising in the leg.

6. High-grade B-cell lymphoma (HGBCL) NOS: Mature B-cell lymphoma that does not fulfil the diagnostic criteria for other defined lymphoma entities.

Definitive Diagnostic Methods

Clinical diagnosis
Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

Alterations in TP53 and CCND2 (Richter syndrome)
Amplifications of BCL2 and MALT1 (DLBCL, leg type)
MYC rearrangements
MYC and BCL2 rearrangements
MYC and BCL6 rearrangements

Immunophenotyping

BCL2+ (expression/positive)
BCL6- (no expression/negative)
CD5+ (expression/positive)
CD20+ (expression/positive)
CD23+ (expression/positive)
EBER+ (expression/positive) (EBV positive DLBCL)
LEF1+ (expression/positive)
PD1+ (expression/positive)
IRF4 (MUMI)+ (expression/positive) (DLBCL, leg type)
MYC+ (expression/positive)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Radiation therapy

Transformations to

None

Transformations from

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C83.3 Non-Hodgkin lymphoma large cell (diffuse)
C83.9 Diffuse non-Hodgkin lymphoma, unspecified
C83.6 Non-Hodgkin lymphoma undifferentiated (diffuse)

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

C83.3_ Diffuse large B-cell lymphoma (effective October 01, 2015)
C83.3A Diffuse large B-cell lymphoma, in remission (effective October 01, 2024)
C83.9_ Non-follicular (diffuse) lymphoma, unspecified (effective October 01, 2015)
C83.90 Primary central nervous system lymphoma (effective October 01, 2024)
C83.9A Non-follicular (diffuse) lymphoma, unspecified, in remission (effective October 01, 2024)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Rapidly enlarging mass at single or multiple nodal or extranodal sites
Skin rash or itchy skin
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
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Age: 70 years median age (occur in children and adults)
Incidence: 25-30% of adult NHL's (western countries)
Sex: slight male predominance
Survival: 5 year survival ~62% without bone marrow involvement; 5 year survival 10% with bone marrow involvement

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: Large B-cell lymphomas
Pages: Part B: 463-472, 476-480, 493-499, 509-519, 530-531

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 Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <08/22/2024>. Available at: https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq. Accessed <03/19/2025>. [PMID: 26389337]
Section: Aggressive B-Cell Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/aggressive-b-cell-lymphoma-treatment-pdq
Glossary