Search Database ICD-O-3 Code Lists

Name

Plasmablastic lymphoma (PBL)

ICD-O-3 Morphology

9735/3: Plasmablastic lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 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

See abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

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

Plasmablastic lymphoma usually involves extranodal sites, such as the nasal/oral cavity (gingiva and palate most common), digestive system, bone and soft tissues, and skin. Nodal involvement can also occur without apparent extranodal disease.

Most causes occur in the setting of immune deficiency/ dysregulation (e.g., HIV infection).

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

None

Definition

Plasmablastic lymphoma (PBL) is an aggressive lymphoid neoplasm composed of large, atypical B cells with plasmablastic or immunoblastic morphology and a terminal B-cell differentiation phenotype, predominantly arising at extranodal sites. (WHO 5th ed)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

CARD11 mutations
MYC translocation (more common in EBV+ cases)
MYC rearrangements, usually with IG genes
TP53 mutations

Immunophenotyping

Blimp1+ (expression/positive)
CD20+/- (weakly positive/negative)
CD38+ (expression/positive)
CD45 (LCA) +/- (weakly positive/negative)
CD79a+ (expression/positive)
CD138+ (expression/positive)
IRF4/MUM1+ (expression/positive)
Ki67 proliferation index usually very high
PAX5+/- (weakly positive/negative)
VS38c+ (expression/positive)
XBP1+ (expression/positive)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Radiation therapy

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

Disseminated bone involvement
Drenching night sweats
Fatigue
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

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: 50 years median age (mostly adults, rare in children)
Incidence: rare disease, most common lymphoma in HIV patients
Sex: M:F ratio: 3:1
Survival: 6-32 months

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: Large B-cell lymphomas
Pages: Part B: 506-508

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