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

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

6 - B-cell
Module Rule
Alternate Names
HHV8-positive plasmablastic lymphoma
Kaposi Sarcoma herpes virus (KSHV)-positive plasmablastic lymphoma
This disease is composed of HHV8-infected lymphoid cells resembling plasmablasts expressing IgM and arising in the setting of multicentric Castleman disease (MCD). It is usually associated with HIV.
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
Genetics Data
IG genes are unmutated
CD20+ or CD20-
cIgA positive
cIgM negative
Epstein-Barr encoded RNA (EBER)-negative
Nuclear staining for interleukin-6
Nuclear staining for LANA-1
Strongly express cIgM with lambda light chain restriction
Stem cell transplant
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)
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
Epidemiology and Mortality
Country: most common in African and Mediterranean countries