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 Abstractor Notes and Module 7

Help me code for diagnosis year :

Grade
6 - B-cell
Module Rule
None
Alternate Names
Angiotropic large 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. Two major forms: Western, which is predominantly a neurological presentation and Asian, which is predominantly multi-organ failure
Abstractor Notes
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9680/3.) This lymphoma is usually widely disseminated in extranodal sites including bone marrow and may present virtually in any organ. Lymph nodes are usually spared. Two major patterns of clinical presentation have been recognized, a Western form characterized by symptoms related to the main organ involved, predominantly neurological or cutaneous and an 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.

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
None
Genetics Data
Immunoglobulin genes are clonally rearranged
Immunophenotyping
CD5 and CD10 coexpression
CD10- cases are IRF4/MUM1 positive
IRF4/MUM1
Treatments
Chemotherapy
Transformations to
No Transformations
Transformations from
No 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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None