EBV-associated lymphoid lesions Flashcards
Latency types of EBV
Latency I - Hiding program
Latency II - Rescue program
Latency III - Growth program
EBV Latency I immunophenotype
EBNA-1 +
LMP-2A +
EBER +
EBV Latency II immunophenotype
EBNA-1 +
LMP-1 +
LMP-2A +
EBER +
EBV Latency III immunophenotype
ENBA-1 +
EBNA-2 +
(EBNA 4-6 +)
LMP-1 +
LMP-2A +
EBER +
Important stains for typing the EBV latency phenotype
EBER (to confirm infection)
EBNA-2
LMP-1
Distinct EBV+ entities
9 entities in total:
1. Lymphomatoid granulomatosis
2. EBV+ DLBCL
3. Fibrin-associated LBCL
4. DLBCL associated with chronic inflammation
5. Plasmablastic lymphoma
6. EBV+ Mucocutaneous ulcer
7. Hyperplasia, EBV+, IDD-associated
8. Polymorphic LPD, EBV+, IDD-associated
9. B cell lymphoma, EBV+, IDD-associated
Immunodeficiency-independent EBV+ entities
Lymphomatoid granulomatosis
EBV+ DLBCL
These can happen in anyone
Local immune dysreguation-associated EBV+ entities
Fibrin-associated Large B cell Lymphoma
DLBCL associated with chronic inflammation
These are usually unexpected diagnoses that are discovered incidentally in someone presenting with another problem. The patients are usually systemically immunocompetent, but have a longstanding focus off chronic inflammation that results in local immunodysregulation. This may be due to foreign body reaction, indolent tumors, or longstanding fluid collections.
In both cases, cells are large, atypical, EBER+, B cell marker +, MUM1+. The majority of cases are also CD43+, CD30+, and PDL1+, and have high Ki67 indices (>70%, often around 90%).
Etiologically, these are thought to be variations of the same entity. Fibrin-associated is considered a subtype of DLBCL associated with chronic inflammation.
Variably immunodeficinecy-associated EBV+ entities
Plasmablastic lymphoma
Systemic immunodeficieency-associated EBV+ entities
EBV+ mucocutaneous ulcer
Hyperplasia, EBV+, IDD-associated
Polymohpric lymphoproliferative disorder, EBV+, IDD-associated
B-cell lymphoma, EBV+, IDD-associated
How to define “B-cell lymphoma, EBV+, IDD-associated”
This is a diagnosis that you make to trump other diagnoses if the tumor meets the two criteria (EBV+, in the setting of systemic IDD).
If these criteria are met, you would make this diagnosis INSTEAD of:
Burkitt Lymphoma, EBV+
Classic Hodgkin Lymphoma
Marginal Zone Lymphoma
Follicular Lymphoma
Found incidentally in tissue from a knee joint arthroplasty
Fibrin-associated DLBCL
Belongs to a group of two related diagnoses: DLBCL a/w CI and FA-DLBCL
These are usually unexpected diagnoses that are discovered incidentally in someone presenting with another problem. The patients are usually systemically immunocompetent, but have a longstanding focus off chronic inflammation that results in local immunodysregulation. This may be due to foreign body reaction, indolent tumors, or longstanding fluid collections.
In both cases, cells are large, atypical, EBER+, B cell marker +, MUM1+. The majority of cases are also CD43+, CD30+, and PDL1+, and have high Ki67 indices (>70%, often around 90%).
Etiologically, these are thought to be variations of the same entity. Fibrin-associated is considered a subtype of DLBCL associated with chronic inflammation.
Polypoid left atrial mass resection
DLBCL associated with chronic inflammation
Belongs to a group of two related diagnoses: DLBCL a/w CI and FA-DLBCL
These are usually unexpected diagnoses that are discovered incidentally in someone presenting with another problem. The patients are usually systemically immunocompetent, but have a longstanding focus off chronic inflammation that results in local immunodysregulation. This may be due to foreign body reaction, indolent tumors, or longstanding fluid collections.
In both cases, cells are large, atypical, EBER+, B cell marker +, MUM1+. The majority of cases are also CD43+, CD30+, and PDL1+, and have high Ki67 indices (>70%, often around 90%).
Etiologically, these are thought to be variations of the same entity. Fibrin-associated is considered a subtype of DLBCL associated with chronic inflammation.
LMP-2A
Decoy B-cell receptor
The key protein involved in the “rescue program.”
Saves B cells which fail BCR rearrangement from apoptosis.
EBV+ Mucocutaneous Ulcer
EBV+ lymphoproliferative disorder which occurs in the oropharyngeal mucosa of patients with immunosuppression (usually iatrogenic or age-related senescence).
Contains a polymorphic hematolymphoid infiltrate that includes large EBV-positive B cells and variable Hodgkinoid cells in a background rich in T cells.
EBER+ by definition, typically CD30+ and pan-B-cell marker positive, usually CD15 negative.
