Plasmablastic lymphoma

Name
ICD-O-3 Morphology
9735/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
See Module 7
Most common sites of involvement: oral cavity mass, mucosal sites, sinonasal cavity, orbit, skin, bone, soft tissues and GI tract. For HIV negative patients-lymph nodes

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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.

Most patients present at Stages III/IV. The international prognostic index (IPI) is of intermediate to high risk.

EBV positivity is seen in the majority of patients.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Clonal IgH light chain rearrangement
Immunophenotyping
CD20 weakly positive
CD30+
CD38+
CD45 weakly positive
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
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
C83.3 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
Disseminated bone involvement
Drenching night sweats
Fatigue
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
Bone marrow aspiration and biopsy
CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Progression and Transformation
None
Epidemiology and Mortality
Age: 50 years median age (mostly adults, rare in children)
Incidence: rare disease, most common lymphoma in HIV patients
Sex: male predominance
Survival: <1 year