Name

Intravascular large B-cell lymphoma

ICD-O-3 Morphology

9712/3: Intravascular large B-cell lymphoma
Effective 2010 and later

Reportable

for cases diagnosed 2010 and later

Primary Site(s)

See Module 7
Usually widely disseminated in extranodal sites, including the bone marrow. May present in any organ. Lymph nodes are usually spared.

Grade

Not Applicable

Module Rule

None

Alternate Names

Angioendotheliomatosis
Angioendotheliomatosis proliferans syndrome [OBS]
Angioendotheliotropic lymphoma [OBS]
Angiotropic large cell lymphoma
Angiotropic lymphoma
Intravascular B-cell lymphoma
Intravascular lymphomatosis [OBS]
IVLBCL
Malignant angioendotheliomatosis [OBS]

Definition

Intravascular large B-cell lymphoma is a rare type of extranodal large B-cell lymphoma characterized by the selective growth of lymphoma cells within the lumina of vessels, in particular capillaries, and with the exception of larger arteries and veins.

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9680/3.)

1. Western form characterized by symptoms related to the main organ involved, predominantly neurological or cutaneous

2. Asian variant in which the patients present with multi-organ failure, hepatosplenomegaly, pancytopenia, and hemophagocytic syndrome. B symptoms very common in both types of presentations. This is an aggressive lymphoma which responds poorly to chemotherapy. The poor prognosis reflects in part frequent delays in diagnosis because of a lack of detectable tumor masses.

An isolated cutaneous variant has been identified invariably in Western females. It is characterized by limitation of tumor to the skin and has a better prognosis.

Definitive Diagnostic Methods

Genetic testing
Immunophenotyping

Genetics Data

Immunoglobulin genes are clonally rearranged

Immunophenotyping

CD5 and CD10 coexpression
CD10- cases are IRF4/MUM1 positive
CD29 (inegrin beta-1) lacking
CD54 (ICAM1) adhesion beta-molecules lacking

Treatments

Chemotherapy

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

C83.8 Other types of diffuse non-Hodgkin's lymphoma

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

C83.8 Other non-follicular lymphoma (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
Fever (for no known reason)
Hemaphagocytic syndrome
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

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

Progression and Transformation

None

Epidemiology and Mortality

Age: 67 years median age (13-85 years range)
Incidence: differs according to geographic origin of patients between West and East
Sex: no male or female predominance

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: 317-318

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