Search Database ICD-O-3 Code Lists

Name

KSHV/HHV8-positive diffuse large B-cell lymphoma

ICD-O-3 Morphology

9738/3: Large B-cell lymphoma arising in HHV8 associated multicentric Castleman disease
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Module 7
Most common sites of involvement: lymph nodes (C770-C779)
Common metastatic site: peripheral blood/bone marrow
Spleen involvement is common due to dissemination of disease

Assign C779 if specific primary site cannot be determined.

See abstractor notes.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

KSHV/HHV8-positive diffuse large B-cell lymphoma 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)

KSHV/HHV8-positive DLBCL characteristically involves the lymph nodes. Dissemination to the spleen is common, but it can also disseminate to extranodal sites and can also manifest with peripheral blood involvement. Rarely, the lymphoma is limited to the spleen.

Most cases are positive for HIV.

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

Large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease (HHV8 MCD)

Definition

Kaposi sarcoma–associated herpesvirus / human herpesvirus 8 (KSHV/HHV8)-positive diffuse large B-cell lymphoma (DLBCL) is a large B-cell lymphoma consistently associated with KSHV/HHV8, in general arising in patients with profound immunodeficiency, but it may occur in non-immunocompromised patients. The tumour cells morphologically resemble plasmablasts or immunoblasts and have abundant cytoplasmic IgM. (WHO 5th ed)

Definitive Diagnostic Methods

Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

None

Immunophenotyping

IgM producing cells without IG somatic hypermutations
LANA (KSHV/HHV8)+ (expression/positive)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy
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)

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
Enlarged lymph nodes
Fatigue
Fever (for no known reason)
Manifestations of Kaposi Sarcoma
Massive splenogmegaly
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

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: HIV positive men between aged 30 and 40 years
Country: most common in African and Mediterranean countries
Incidence: very rare
Sex: Male predominance
Survival: poor

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: KSHV/HHV8-associated B-cell lymphoid proliferations and lymphomas
Pages: Part B: 545-546

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 <03/20/2025>.
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