Epstein-Barr virus-associated T- and NK-cell lymphoproliferative diseases: an update and diagnostic approach.
Pathology
; 52(1): 111-127, 2020 Jan.
Article
in En
| MEDLINE
| ID: mdl-31767131
ABSTRACT
Epstein-Barr virus (EBV)-positive T-cell and natural killer (NK)-cell lymphoproliferative diseases (EBV-TNKLPD) are a group of uncommon disorders characterised by EBV infection of T- and NK-cells. As a group, EBV-TNKLPD are more commonly encountered in Asians and Native Americans from Central and South America compared to Western populations. They encompass a spectrum of entities that range from non-neoplastic lesions such as EBV-associated haemophagocytic lymphohistiocytosis (EBV-HLH) to more chronic conditions with variable outcomes such as chronic active EBV infections (CAEBV) of T- and NK-cell type (cutaneous and systemic forms) and malignant diseases such as systemic EBV-positive T-cell lymphoma of childhood, aggressive NK-cell leukaemia, extranodal NK/T-cell lymphoma, nasal-type, and primary EBV-positive nodal T/NK-cell lymphoma. Due to their rarity, broad clinicopathological spectrum and significant morphological and immunophenotypic overlap, the diagnosis and precise classification of EBV-TNKLPD often pose a challenge to clinicians and pathologists. Correct classification of this group of rare diseases relies heavily on the age of onset, disease presentation, duration of symptoms and cell of origin (T- vs NK-cell lineage). In this review, we provide an update on the clinicopathological and molecular features of the various EBV-TNKLPD entities occurring in non-immunocompromised patients and present a practical algorithmic approach for the general pathologist who is confronted with these disorders in routine clinical practice.
Key words
EpsteinBarr virus-positive T-cell and NK-cell lymphoproliferative disease; Hydroa vacciniforme-like lymphoproliferative disorder; aggressive NK-cell leukaemia; childhood; chronic active EBV infection; differential diagnosis; extranodal NK/T cell lymphoma; haemophagocytic lymphohistiocytosis; peripheral T-cell lymphoma; severe mosquito bite allergy
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Killer Cells, Natural
/
Lymphoma, T-Cell
/
Herpesvirus 4, Human
/
Epstein-Barr Virus Infections
/
Lymphoproliferative Disorders
Type of study:
Diagnostic_studies
/
Risk_factors_studies
Limits:
Humans
Language:
En
Journal:
Pathology
Year:
2020
Document type:
Article
Affiliation country:
Singapore