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

被引：357

作者：

Kimura, Hiroshi ^{[1
]}

Ito, Yoshinori ^{[2
]}

Kawabe, Shinji ^{[2
]}

Gotoh, Kensei ^{[2
]}

Takahashi, Yoshiyuki ^{[2
]}

Kojima, Seiji ^{[2
]}

Naoe, Tomoki ^{[3
]}

Esaki, Shinichi ^{[4
,5
]}

Kikuta, Atsushi ^{[6
]}

Sawada, Akihisa ^{[7
,8
]}

Kawa, Keisei ^{[7
,8
]}

Ohshima, Koichi ^{[9
]}

Nakamura, Shigeo ^{[10
]}

机构：

[1] Nagoya Univ, Dept Virol, Grad Sch Med, Showa Ku, Nagoya, Aichi 4668550, Japan

[2] Nagoya Univ, Dept Pediat, Grad Sch Med, Nagoya, Aichi 4668550, Japan

[3] Nagoya Univ, Dept Hematol, Grad Sch Med, Nagoya, Aichi 4668550, Japan

[4] Nagoya City Univ, Dept Otolaryngol Head & Neck Surg, Grad Sch Med Sci, Nagoya, Aichi, Japan

[5] Sch Med, Nagoya, Aichi, Japan

[6] Fukushima Med Univ, Div Pediat Oncol, Ctr Canc, Fukushima, Japan

[7] Osaka Med Ctr, Dept Pediat, Osaka, Japan

[8] Res Inst Maternal & Child Hlth, Osaka, Japan

[9] Kurume Univ, Sch Med, Dept Pathol, Kurume, Fukuoka 830, Japan

[10] Nagoya Univ Hosp, Dept Pathol & Lab Med, Nagoya, Aichi, Japan

来源：

BLOOD | 2012年 / 119卷 / 03期

关键词：

EPSTEIN-BARR-VIRUS; VACCINIFORME-LIKE ERUPTION; HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; HYDROA VACCINIFORME; VIROLOGICAL CHARACTERISTICS; VIRAL-DNA; T-CELLS; INFECTION; LYMPHOMA; DISORDERS;

D O I：

10.1182/blood-2011-10-381921

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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. (Blood. 2012;119(3): 673-686)

引用

页码：673 / 686

页数：14