Name

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

Reportable

for cases diagnosed 2010 and later

Primary Site(s)

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

Grade

Not Applicable

Module Rule

None

Alternate Names

HHV8 MCD
HHV8 PL
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

Definition

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.

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.

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

IG genes are unmutated

Immunophenotyping

CD20 positive or negative
CD27-
CD38 positive or negative
CD79a-
CD138-
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

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy
Surgery

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

202.8 Other lymphoma

Corresponding ICD-10 Codes

C85.7 Other specified types of non-Hodgkin's lymphoma

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

C85.8 Other specified types of non-Hodgkin 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

CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Country: most common in African and Mediterranean countries

Sources

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, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary