Primary effusion lymphoma

Name
ICD-O-3 Morphology
9678/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
See Module 7
Most common sites of involvement: body cavities (pleural, pericardial, and peritoneal). Extracavity lesions may occur in: GI tract, skin, lung, and CNS, and lymph nodes.

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 (typically only one body cavity is involved). Because there are no ICD-O-3 codes for the pleural space, pericardium, or peritoneal cavity, code the primary site to:
1. Pleura (C384) when it occurs in the pleural cavity
2. Pericardium (C380) when it occurs in the pericardium
3. Peritoneum (C482) when it occurs in the peritoneal cavity.

PEL is generally resistant to cancer chemotherapy drugs that are active against other lymphomas, and carries a poor prognosis.
Definitive Diagnostic Methods
Cytology
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Immunoglobulin genes clonally rearranged and hypermutated
Immunophenotyping
BCL6 absent
CD19-
CD20-
CD30 (often demonstrable)
CD38 (often demonstrable)
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
Drenching night sweats
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Weight loss (for no known reason)
Diagnostic Exams
CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic reasonance imaging)
PET (positron emission tomography) scan
Progression and Transformation
None
Epidemiology and Mortality
Age: young or middle aged HIV patients
Sex: male predominance
Survival: <6 months