Primary effusion lymphoma

Name
Primary effusion lymphoma
ICD-O-3 Morphology
9678/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 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
Body-cavity based lymphoma
PEL
Definition
Aggressive B-cell lymphoma usually confined to pleural, pericardial, peritoneal body cavities, presenting as serous effusions without detectable tumor masses, occurring primarily but not exclusively in HIV-infected patients, caused by gamma 2 herpes virus (Kaposi sarcoma-associated herpes virus KSHV, aka human herpes virus type 8, HHV-8)
Abstractor Notes
PEL is universally associated with human herpes virus 8 (HH8).

PEL is unusual in that the majority of cases arise in body cavities, such as the pleural, pericardial, and peritoneal cavities. Because there are no ICD-O-3 codes for the pleural space, pericardium, or peritoneal cavity, code the primary site to pleura C384 when the neoplasm arises in the pleural cavity, to pericardium C380 when it occurs in the pericardium, and to peritoneal cavity C482 when it occurs in the peritoneum. Typically only one body cavity is involved. Extracavitary tumors with morphologic and phenotypic characteristics similar to PEL occur in extranodal sites including the GI tract, skin, lung, and CNS, or may involve lymph nodes.

PEL is generally resistant to cancer chemotherapy drugs that are active against other lymphomas, and carries a poor prognosis.
Definitive Diagnostic Methods
Cytology
Histology
Genetics Data
Immunoglobulin genes clonally rearranged and hyperstimulated
Immunophenotyping
BCL6 absent
CD19-
CD20-
CD30
CD38
CD45+
CD79a-
HLA-DR
LANA(ORF 73)
Surface and cytoplasmic immunoglobulin absent
Treatments
Chemotherapy
Other RX
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
200.8 Other named variants, 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