EBV-associated T/NK-cell lymphoproliferative diseases in nonimmunocompromised hosts: prospective analysis of 108 cases.
نویسندگان
چکیده
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.
منابع مشابه
CLINICAL TRIALS AND OBSERVATIONS EBV-associated T/NK–cell lymphoproliferative diseases in nonimmunocompromised hosts: prospective analysis of 108 cases
Departments of 1Virology, 2Pediatrics, and 3Hematology, Nagoya University Graduate School of Medicine, Nagoya, Japan; 4Department of Otolaryngology, Head and Neck Surgery, Nagoya City University Graduate School of Medical Sciences and Medical School, Nagoya, Japan; 5Division of Pediatric Oncology, Fukushima Medical University Cancer Center, Fukushima, Japan; 6Department of Pediatrics, Osaka Med...
متن کاملEpstein–barr Virus (ebv)-associated T/nk Lymphoproliferative Diseases in Non-immunocompromised Hosts: Prospective Analysis of 108 Cases
Affiliations Departments of Virology, Pediatrics and Hematology, Nagoya University Graduate School of Medicine, Nagoya, Department of Otolaryngology, Head and Neck Surgery, Nagoya City University Graduate School of Medical Sciences and Medical School, Nagoya, Division of Pediatric Oncology, Fukushima Medical University Cancer Center, Fukushima, Department of Pediatrics, Osaka Medical Center and...
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ورودعنوان ژورنال:
- Blood
دوره 119 3 شماره
صفحات -
تاریخ انتشار 2012