HHV8-positive diffuse large B-cell lymphoma, NOS

ICD-O-3 Morphology

9738/3: Large B-cell lymphoma arising in HHV8 associated multicentric Castleman disease
Effective 2010 and later


for cases diagnosed 2010 and later

Primary Site(s)

See Module 7
Most common sites of involvement: lymph nodes (C770-C779)

Abstractor Notes

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

The lymphoma characteristically involves lymph nodes and spleen but can disseminate to other viscera via the blood stream in which rarely it manifests as a leukemia.

The term "plasmablastic" is used for this lymphoma because the cells resemble plasma cells and have abundant immunoglobulin, but they lack IG somatic hyper-mutation.

This lymphoma is considered to be the same primary with the following:
1. Plasmablastic lymphoma (9735/3). The two diagnosis can be distinguished because plasmablastic lymphoma frequently show class-switched and hyper-mutated IG genes.

2. Primary effusion lymphoma (9678/3) may complicate HHV8 MCD but PEL neoplastic cells do no express immunoglobulin and are usually co-infected with EBV.

This is a very aggressive lymphoma with a median survival of a few months.

Note: Castleman disease by itself is not reportable.

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.


Not Applicable

Module Rule


Alternate Names

HHV8-positive DLBCL, NOS
HHV8-positive plasmablastic lymphoma
Kaposi Sarcoma herpes virus (KSHV)-positive plasmablastic lymphoma
Large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease


HHV8-positive diffuse large B-cell lymphoma, NOS, usually arises in association with HHV8-positive multicentric Castleman disease (MCD). The lymphoma is characterized by a monoclonal proliferation of HHV8-infected lymphoid cells resembling plasmablasts expressing IgM lambda. It is usually associated with HIV infection.

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation

Genetics Data

IG genes are unmutated


CD20 positive or negative
CD38 positive or negative
cIgA positive
cIgM negative
cIgM with lambda light chain restriction strongly expressed
Epstein-Barr encoded RNA (EBER)-negative
Nuclear staining for interleukin-6
Nuclear staining for LANA-1


Hematologic Transplant and/or Endocrine Procedures

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

200.7 Large cell lymphoma

Corresponding ICD-10 Codes

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)
D47.Z2 Castleman disease (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
Enlarged lymph nodes
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
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
PET (positron emission tomography) scan

Progression and Transformation


Epidemiology and Mortality

Country: most common in African and Mediterranean countries


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: 327-328

International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)