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EBV-Positive B-Cell Proliferations of Varied Malignant Potential: 2015 SH/EAHP Workshop Report-Part 1
Yasodha Natkunam1, John R Goodlad2, Amy Chadburn3
1From the Stanford University School of Medicine, Stanford, CA.
Insights
B-cell proliferations in immunodeficiency vary in malignant potential. Epstein-Barr virus (EBV) is key for diagnosing some conditions, and localized forms can arise in immunocompetent individuals.
Area of Science:
- Hematopathology
- Immunology
- Oncology
Background:
- B-cell proliferations present diverse malignant potential in immunodeficiency.
- Understanding these conditions is crucial for accurate diagnosis and management.
Purpose of the Study:
- To review B-cell proliferations of varied malignant potential associated with immunodeficiency.
- To establish consensus diagnoses and explore disease definitions.
Main Methods:
- A Workshop Panel reviewed cases of B-cell hyperplasias, lymphoproliferative disorders, and EBV-positive mucocutaneous ulcers.
- Consensus diagnoses were rendered, focusing on disease boundaries and EBV association.
Main Results:
- B-cell proliferations occurred across all immunodeficiency backgrounds.
- Early presentation, younger age, and regression with reduced immunosuppression were characteristic.
- Epstein-Barr virus (EBV) positivity was essential for diagnosing some hyperplasias.
Conclusions:
- A spectrum of B-cell proliferations shows similarities across immunodeficiency backgrounds.
- Localized forms in immunocompetent patients may result from chronic immune stimulation and exhibit indolent behavior.
Objectives:
The 2015 Workshop of the Society for Hematopathology/European Association for Haematopathology aimed to review B-cell proliferations of varied malignant potential associated with immunodeficiency.
Methods:
The Workshop Panel reviewed all cases of B-cell hyperplasias, polymorphic B-lymphoproliferative disorders, Epstein-Barr virus (EBV)-positive mucocutaneous ulcer, and large B-cell proliferations associated with chronic inflammation and rendered consensus diagnoses. Disease definitions, boundaries with more aggressive B-cell proliferations, and association with EBV were explored.
Results:
B-cell proliferations of varied malignant potential occurred in all immunodeficiency backgrounds. Presentation early in the course of immunodeficiency and in younger age groups and regression with reduction of immunosuppression were characteristic features. EBV positivity was essential for diagnosis in some hyperplasias where other specific defining features were absent.
Conclusions:
This spectrum of B-cell proliferations show similarities across immunodeficiency backgrounds. Localized forms of immunodeficiency disorders arise in immunocompetent patients most likely due to chronic immune stimulation and, despite aggressive histologic features, often show indolent clinical behavior.
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