EBV-associated T/NK-cell lymphoproliferative diseases in nonimmunocompromised hosts: prospective analysis of 108 cases.

Abstract

EBV-associated T/NK-cell lymphoproliferative disease (T/NK-LPD) is defined as a systemic illness characterized by clonal proliferation of EBV-infected T or NK cells. We prospectively enrolled 108 nonimmunocompromised patients with this disease (50 men and 58 women; median onset age, 8 years; age range, 1-50 years) evidenced by expansion of EBV(+) T/NK cells in the peripheral blood; these were of the T-cell type in 64 cases and of the NK-cell type in 44, and were clinically categorized into 4 groups: 80 cases of chronic active EBV disease, 15 of EBV-associated hemophagocytic lymphohistiocytosis, 9 of severe mosquito bite allergy, and 4 of hydroa vacciniforme. These clinical profiles were closely linked with the EBV(+) cell immunophenotypes. In a median follow-up period of 46 months, 47 patients (44%) died of severe organ complications. During the follow-up, 13 patients developed overt lymphoma or leukemia characterized by extranodal NK/T-cell lymphoma and aggressive NK-cell leukemia. Fifty-nine received hematopoietic stem cell transplantation, 66% of whom survived. Age at onset of disease (≥ 8 years) and liver dysfunction were risk factors for mortality, whereas patients who received transplantation had a better prognosis. These data depict clinical characteristics of systemic EBV(+) T/NK-LPD and provide insight into the diagnostic and therapeutic approaches for distinct disease.

• Authors: Hiroshi Kimura, Yoshinori Ito, Shinji Kawabe, Kensei Gotoh, Yoshiyuki Takahashi, Seiji Kojima, Tomoki Naoe, Shinichi Esaki, Atsushi Kikuta, Akihisa Sawada, Keisei Kawa, Koichi Ohshima, Shigeo Nakamura
• Journal: Blood (Blood) Vol. 119 Issue 3 Pg. 673-86 (Jan 19 2012) ISSN: 1528-0020 [Electronic] United States
• PMID: 22096243 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• DNA, Viral

Topics

• Adolescent
• Adult
• Child
• Child, Preschool
• DNA, Viral (genetics)
• Epstein-Barr Virus Infections (complications, pathology, virology)
• Female
• Follow-Up Studies
• Herpesvirus 4, Human (genetics, immunology, isolation & purification)
• Humans
• Immunocompromised Host
• Infant
• Killer Cells, Natural (immunology, pathology, virology)
• Lymphoproliferative Disorders (etiology, mortality, pathology)
• Male
• Middle Aged
• Polymerase Chain Reaction
• Prognosis
• Prospective Studies
• Survival Rate
• Young Adult