Search Database ICD-O-3 Code Lists

Name

Intravascular (large) B-cell lymphoma (IVLBCL) (see 9680/3 prior to 2010)

ICD-O-3 Morphology

9712/3: Intravascular large B-cell lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Module 7
Skin is the most common site but may present in any organ. Lymph nodes usually spared.
Common metastatic site: bone marrow

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 9680/3.)

Intravascular (large) B-cell lymphoma (IVLBCL) is part of the Mature B-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B15)

Intravascular large B-cell lymphoma may involve virtually any organ. Lymph nodes are usually spared.

Cutaneous subtype
Skin is the most common site of presentation. Skin presentation includes peau d'orange-like lesions, marked blue-red discoloration, plaques, nodules and tumors.

Hemophagocytic subtype
Presenting symptoms are dominated by those related to haemophagocytic syndrome, hepatosplenomegaly, and thrombocytopenia (> 75%). Bone marrow and peripheral blood evaluation may show tumour cells. The onset and clinical course is very rapid.

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

Angioendotheliomatosis (proliferans syndrome)
Angiotropic large cell lymphoma [OBS]
Classic intravascular (large) B-cell lymphoma (Classic IVLBCL)
Cutaneous intravascular (large) B-cell lymphoma (Cutaneous IVLBCL)
Hemophagocytic intravascular (large) B-cell lymphoma (Hemophagocytic IVLBCL)
Intravascular B-cell lymphoma (see 9680/3 prior to 2010)

Definition

Intravascular large B-cell lymphoma (IVLBCL) is an aggressive extranodal B-cell lymphoma characterized by the proliferation of large neoplastic B cells virtually exclusively within the lumina of blood vessels. (WHO 5th ed)

Definitive Diagnostic Methods

Clinical diagnosis
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

Immunoglobulin genes are clonally rearranged

Immunophenotyping

CD5 and CD10+ (expression/positive)
CD10- cases are IRF4/MUM1+ (expression/positive)
CD29 (inegrin beta-1) lacking
CD54 (ICAM1) (lacking)

Treatments

Chemotherapy

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)

C83.8 Other types of diffuse non-Hodgkin lymphoma

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

C83.8_ Other non-follicular lymphoma (effective October 01, 2015)
C83.8A Other non-follicular lymphoma, in remission (effective October 01, 2024)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Hemaphagocytic syndrome
Hepatosplenomegaly
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Pancytopenia
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 studies
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Age: Median 59-70
Incidence: 0.095 per million
Sex: no male or female predominance
Survival: poor prognosis

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: 520-522

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