Plasmablastic lymphoma

Name
Plasmablastic lymphoma
ICD-O-3 Morphology
9735/3
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
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
None
Alternate Names
PBL
Definition
This is a neoplasm with diffuse proliferation of large neoplastic cells, most of which resemble B immunoblasts, but in which all tumor cells have the immunophenotype of plasma cells. This disease is uncommon. The highest percentage occurs in patients who are HIV positive.
Abstractor Notes
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9684/3.) PBL is uncommon. The highest incidence is in HIV-positive individuals. Usually presents as a mass in the oral cavity but also occurs in other extranodal sites particularly mucosal sites. Other primary sites include the sinonasal cavity, orbit, skin, bone, soft tissues and GI tract. Nodal involvement is uncommon. Most patients present at Stages III/IV. CT and PET scans may show disseminated bone involvement. The international prognostic index (IPI) is of intermediate to high risk. Patients who are HIV negative present more commonly in lymph nodes. EBV positivity is seen in the majority of patients.
Definitive Diagnostic Methods
Histologic confirmation
Immunophenotype
Genetics Data
Clonal IgH light chain rearrangement
Immunophenotyping
CD20 weakly positive
CD30+
CD45 weakly positive
CD38+
CD56- in oral mucosal primary
CD79a+
CD138+
Cytoplasmic immunoglobulins expressed, most frequently IgG and either kappa or lambda light chain
EBV+
EMA+
IRF4/MUM1
Ki67 index very high
PAX5 weakly positive
Vs38c
Treatments
Chemotherapy
Radiation
Stem cell transplant
Transformations to
No Transformations
Transformations from
No Transformations
Corresponding ICD-9 Codes
200.7 Large cell lymphoma
Corresponding ICD-10 Codes
C83.3 Diffuse non-Hodgkins lymphoma, large cell (diffuse)
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C83.3 Diffuse large B-cell lymphoma
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None