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K. Kawamoto et al.
with the NK-cell type had significantly more skin lesions (P=0.0119). A comparison of the clinicopathological fea- tures of CAEBV with nodal lesions versus CAEBV with extranodal lesions is shown in Online Supplementary Table S2. There were no clinicopathological differences between adult-onset CAEBV with nodal lesions and those with extranodal lesions. In addition, the anti-viral capsid antigen
(VCA)-IgM antibody tended to be low or less than the level of detection. The median count of EBER-positive cells was 53 per high power field (range, 2–487), and 86.3% (44/51) of the cases showed ten or more positive cells per high power field. Furthermore, 97.3% (36/37) of the cases had ≥102 EBV copies/mL, and only 2.7% (1/37) had levels below that of the level of detection (2×102 copies/mL).
Table 2. Characteristics of patients aged over 50 years with adult-onset chronic active Epstein-Barr virus infection.
All patients Infected-cell type Patients’ characteristics T-cell
NK-cell P§ (n=22) (n=10) (n=12)
Sex
Male, n (%)/female, n (%)
Symptoms and involved sites Fever, n (%)
Bone marrow, n (%) Splenomegaly, n (%) Hepatomegaly, n (%) Lymphadenopathy, n (%) Skin rash, n (%)
Abdominal disturbance, n (%) Laryngopharynx, n (%)
Lung, n (%)
Gastrointestinal tract, n (%) Orallesion,n(%)
Central nervous system, n (%)
Myocarditis, n (%)
Past medical history
Hypersensitivity to mosquito bites, n (%) Hydroa vacciniforme, n (%)
ECOG PS high (2-4), n (%)
Laboratory test at initial diagnosis Anemia (Hb <10.5 g/dL), n (%) Thrombocytopenia (< 100×109/L), n (%) LDH elevation, n (%)
Transaminase, elevation, n (%) Hemophagocytic syndrome, n (%)
EBV-related antibody to: VCA-IgG, median titer (range) VCA-IgM, median titer (range) EBNA, median titer (range) Unknown, n (%)
EBV-DNA, in plasma (copy/mL) Median (range)
Unknown, n (%)
EBV monoclonality by Southern blot, n (%)
EBER+cell counts /HPF, median (range)
Histological classification9 A1, n (%)
A2, n (%)
A3, n (%)
Immunophenotype CD4
CD8
CD56
TCRab (n = 3)
Allogeneic HSCT
12 (54.5)/10 (45.5)
9 (40.9) 8 (36.4) 7 (31.8) 5 (22.7) 10 (45.5) 11 (50.0) 3 (13.6) 5 (22.7) 0 (0)
0 (0) 0(0) 0 (0) 0 (0)
2 (9.1) 1 (3.7)
6 (27.3)
6 (27.3) 16 (72.7) 13 (74.1) 4 (18.2) 5 (22.7)
160 (80-5120) <10 (<10-10) 40 (<10-80) 9 (40.9)
1×104(nd-1×106) 13 (59.1)
8/9 (88.9) 87 (2-487)
5 (22.7) 7 (31.8) 10 (45.5)
10 (45.5) 10 (45.5) 10 (45.5) 3/3 (100)
2 (9.1)
5 (50.0)/5 (50.0)
5 (55.6) 4 (50.0) 4 (57.1) 4 (80.0) 7 (70.0) 2 (18.2) 1 (33.3) 2 (40.0) 0 (0)
0 (0) 0(0) 0 (0) 0 (0)
0 (0) 0 (0)
4 (66.7)
3 (50.0) 5 (31.3) 6 (72.7) 3 (75.0) 4 (80.0)
160 (10-1280) <10 (<10-10) 10 (<10-40) 3 (33.3)
2×105(5×104-1×106) 7 (53.8)
3/4 (75.0) 58 (2-435)
3 (60.0) 3 (42.9) 4 (40.0)
7 (70.0) 4 (40.0) 5 (50.0) 3/3 (100)
1 (50.0)
7 (58.3)/5 (41.7) 1
4 (44.4)
4 (50.0) 1
0.666
3 (42.9)
1 (20.0)
3 (30.0)
9 (81.8) 0.03 2 (66.7) 1 3 (60.0) 1
0 (0) na 0 (0) na 0(0) na 0 (0) na 0 (0) na
0.652 0.135 0.0212
2 (100)
1 (100) 1
11 (68.7)
7 (75.0) 1
1 (25.0) 1 (20.0)
0.293 0.135
0.481
2 (33.3)
3 (50.0) 1
0.348
0.0557
160 (80-5120) na <10 (<10-10)
40 (<10-80)
6 (66.7)
1.1×103(nd-7.3×104) na 6 (46.2)
5/5 (100) na
1022
168 (15-487)
2 (40.0) 4 (57.1) 6 (60.0)
0.308
0.864
3 (30.0) na 6 (60.0)
5 (50.0)
na
1 (50.0) na
CAEBV: chronic active EBV infection; EBV: Eptein-Barr virus; EBNA: Epstein–Barr virus nuclear antigen 1; EBV-DNA: EBV-deoxyribonucleic acid; ECOG PS: Eastern Cooperative OncologyGroupPerformanceStatus;EBER:Epstein-Barrvirus-encodedRNA;nd:notdetected(EBV-DNA<2×102 copies/mL);HPF:highpowerfield;HSCT:hematopoieticstem cell transplantation; Hb:hemoglobin;LDH:lactate dehydrogenase;TCR:T-cell receptor; VCA:viral capsid antigen.*Statistically significant;na:not avalilable;P§:T-cell type versus NK-cell type.
haematologica | 2018; 103(6)