Spectrum of childhood Epstein-Barr virus-associated T-cell proliferations and bone marrow findings.
Systemic Epstein-Barr virus-positive (EBV+) T-cell lymphoproliferative disorder of childhood is a recently described entity. The majority of such cases have been reported from Asia, which suggests an underlying genetic predisposition. We analyzed the clinicopathologic characteristics of 5 children with EBV+ T-cell lymphoid proliferations evaluated and treated at our institute over a 2-year period. There were 3 males and 2 females of Latino (n  =  4) or Caucasian (n  =  1) heritage with a median age of 5 years (age range 2-18 years). All patients presented with EBV infection (acute, n  =  4) with elevated serum EBV viral loads at the time of diagnosis and had systemic manifestations, including fever, hepatosplenomegaly, and pancytopenia. The bone marrow biopsies showed EBV+/CD8+ T-cell lymphocytosis in all patients, with variable degrees of histiocytosis, plasmacytosis, and hemophagocytosis. Interestingly, there was marked and consistent depletion of mature and precursor B cells in the marrow (<1% of total marrow cellularity) in all patients. Three of the patients died of disease-associated complications 2 to 12 weeks after initial diagnosis. Our study describes the detailed bone marrow findings, contributes to the growing number of cases of systemic EBV+ T-cell lymphoproliferative disorder of childhood occurring in the Western hemisphere, and documents this disorder in patients from the Caribbean countries.
Sevilla
Deborah W
DW
Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York, NY, USA.
El-Mallawany
Nader Kim
NK
Emmons
Foxwell N
FN
Alexander
Suzy
S
Bhagat
Govind
G
Alobeid
Bachir
B
D000293:Adolescent; D001853:Bone Marrow / Q000276:immunology / Q000473:pathology*; D002469:Cell Separation; D002675:Child, Preschool; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000276:immunology / Q000473:pathology*; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D016399:Lymphoma, T-Cell / Q000276:immunology / Q000473:pathology* / Q000821:virology*; D008232:Lymphoproliferative Disorders / Q000473:pathology; D008297:Male; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology; D019562:Viral Load