Name

Plasmablastic lymphoma

ICD-O-3 Morphology

9735/3: Plasmablastic lymphoma
Effective 2010 and later

Reportable

for cases diagnosed 2010 and later

Primary Site(s)

See Module 7
Most common sites of involvement: oral cavity mass, mucosal sites, sinonasal cavity, orbit, skin, bone, soft tissues and GI tract. For HIV negative patients-lymph nodes

Grade

Not Applicable

Module Rule

None

Alternate Names

PBL

Definition

Plasmablastic lymphoma (PBL) is a very aggressive lymphoma with a diffuse proliferation of large neoplastic cells, most of which resemble B immunoblasts or plasmablasts, that have a CD20-negative plasmacytic phenotype.

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9684/3.)

PBL is uncommon. The highest incidence is in HIV-positive individuals.

Most patients present at Stages III/IV. The international prognostic index (IPI) is of intermediate to high risk.

EBV positivity is seen in the majority of patients.

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

Clonal IgH light chain rearrangement
MYC translocation (more common in EBV+ cases)

Immunophenotyping

BCL2 and BCL6 expression absent
CD10 expressed
CD20 negative or weakly positive
CD30 expressed
CD38+
CD45 negative or weakly positive
CD56- in oral mucosal primary
CD79a+
CD138+
Cyclin D1 negative
Cytoplasmic immunoglobulins expressed, most frequently IgG and either kappa or lambda light chain
EBV+
EMA expressed
IRF4/MUM1
Ki67 proliferation index usually very high
PAX5 negative or weakly positive
PRDM1 (also called BLIMP1)
VS38c+
XBP1

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Radiation therapy

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 Diffuse non-Hodgkins lymphoma, large cell (diffuse)

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

C83.3 Diffuse large B-cell lymphoma (effective October 01, 2015)

Signs and Symptoms

Disseminated bone involvement
Drenching night sweats
Fever (for no known reason)
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

Progression and Transformation

None

Epidemiology and Mortality

Age: 50 years median age (mostly adults, rare in children)
Incidence: rare disease, most common lymphoma in HIV patients
Sex: male predominance
Survival: <1 year

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: 321-322

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