Intravascular large B-cell lymphoma

Name
Intravascular large B-cell lymphoma
ICD-O-3 Morphology
9712/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
See Module 7
This lymphoma is usually widely disseminated and can occur in any part of the body

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Grade
6 - B-cell
Module Rule
None
Alternate Names
Angioendotheliomatosis
Angiotropic large cell lymphoma
Angiotropic lymphoma
Intravascular B-cell lymphoma
IVLBCL
Definition
This is a rare type of extranodal large B-cell lymphoma characterized by the selective growth of lymphoma cells within the lumina of vessels, particularly capillaries, with 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.)

Lymph nodes are usually spared. Two major patterns of clinical presentation have been recognized:

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
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 (effective October 1, 2015 U.S. only)
C83.8 Other non-follicular lymphoma
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