Name

T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) (see 9680/3 prior to 2010)

ICD-O-3 Morphology

9688/3: T-cell/histiocyte-rich large B-cell lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C770-C779
Lymph nodes (C770-C779) are the usual primary sites; however, involvement in other sites is possible.
Common metastatic sites include the bone marrow/peripheral blood, and liver.
Spleen involvement is common due to dissemination of disease

Assign C779 if specific primary site cannot be determined.

See abstractor notes

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9680/3.)

T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) 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)

T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) usually affects the lymph nodes.

Usually manifests as Stage III-IV systemic disease, typically with B symptoms, splenomegaly, and/or hepatomegaly.

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

Histiocyte-rich large B-cell lymphoma
T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) progressed from nodular lymphocyte predominant Hodgkin lymphoma
T-cell rich (large) B-cell lymphoma
T-cell rich/histiocyte-rich large B-cell lymphoma, de novo

Definition

T-cell/histiocyte–rich large B-cell lymphoma (THRLBCL) is an aggressive B-cell lymphoma with < 10% large neoplastic B cells, scattered in a diffuse background rich in T cells and histiocytes, and with a virtual absence of small B cells. A subset of cases shows marked clinical, immunophenotypic, and molecular overlap with nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL). (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

CD274 (PDL1)/ODCD1/LG2 (gains/amplications)

Immunophenotyping

CD15- (no expression/negative)
CD20+ (expression/positive)
PD1+ T-cells
PDL1+ macrophage surrounding neoplastic B-cell

Treatments

Chemotherapy
Hormone therapy
Radiation therapy

Transformations to

None

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

C83.3 Non-Hodgkin lymphoma large cell (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)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Hepatomegaly
Malaise
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Splenomegaly
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: primary middle age (12-61 year range)
Sex: male predominance

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: 473-475

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 Aggressive B-Cell Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <02/24/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/aggressive-b-cell-lymphoma-treatment-pdq. Accessed <03/19/2025>.
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