Peripheral T Cell Lymphoma with a High Titer of Proteinase-3-Antineutrophil Cytoplasmic Antibodies that Resembled Wegener s Granulomatosis

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1 CASE REPORT Pph T C Lymphm wh Hh T Pn-3-Annuph Cypm Anbd h Rmbd Wn Gnum Tuyh Sh 1, Rk Tkhh 1, Yum Tjm 1, Ku Kh 1, Jj Ymm 1, Hh Fuj 1, Nuhk Tkw 1, Knh Ihzw 1, Ry Ihnhm 2, Tmn Ih 1 nd Hd H 1 Ab H, w pn 54-y-d mn wh pp nd wn bw h yd. Lby ndn hwd hh v PR3-ANCA nd h xmnn h bpy vd u nmmn. Th wh h ndn MRI, dn WG w md. Hwv, h d pd pdy nd h xmnn h nd bpy vd nn np T ymphy wh bn CD7. A n dn pph T ymphm, n hw pd (WHO) w md, nd mp mn w hvd by hmhpy. Th vy T ymphm wh hh PR3-ANCA. Ky wd: Wn num, T ymphm, pn-3-nnuph ypm nbdy () () Indun Sym nmmy d, nudn ummun d, mmny nvv h y uundn u. Fm hum ppv, Wn num (WG) pnv d h h b (1). WG hud b upd n pn wh unxpnd ympm ym nmmy d, d n vu, nd wy mpn wh whu n munph (2). H, w pn pph T ymphm wh hn nu nd pp h mbd WG. Th pn w upd hv WG du hh pn-3-nnuph ypm nbd (PR3-ANCA) nd h h ndn n bpy m h mxy nu hwn ny nmmy hn. Hwv, h d pd pdy nd dn pph T ymphm, n hw pd (WHO) w ny md bd n p bpy ndn. Th n u nd ndn h pn u h mpn u dn dn b um by h xmnn. C Rp A 54-y-d mn hd ud m hn nu n Jnuy 2008, nd undwn uy n My. In md- My, wn ud bw h yd. Lby xmnn hwd hh PR3-ANCA (>500 EU) by nzym-nkd mmunbn y (ELISA) nd h ny bpy m h mxy nu vd nn nmmy wh p umun hy nd num-k hn. H w upd b un m WG bd n by nd h ndn. In Jun, h pp pd nd h vu mpmn wnd h n mvmn v. H w hn d u hp mn. On dmn, pp wh hd m bw h yd Dpmn Hmy nd Rhumy, Thku Unvy Gdu Sh Mdn, Snd nd Dpmn Hmphy, Thku Unvy Gdu Sh Mdn, Snd Rvd pubn Jun 17, 2009; Apd pubn Auu 7, 2009 Cpndn D. Hd H, h@m.n.hku..jp 2041

2 Tb1. LbyFndnnAdmn Uny γ-gtp (IU/L) 56 Pn ChE (IU/L) 256 Ou bd AMY (U/L) 41 WBC (/µl) 6600 TP (/dl) 7.3 S. (%) 64 Ab (/dl) 3.5 Sb. (%) 0 BUN (m/dl) 24 E. (%) 1 C (m/dl) 1.0 B. (%) 1 N (meq/l) 137 Ly. (%) 24 K (meq/l) 4.1 Mn. (%) 11 C (meq/l) 97 RBC (10 4 /µl) 445 SAA (μ/ml) Hb (/dl) 13.6 CRP (m/dl) 12.2 PLT (10 4 /µl) 62.4 IG (m/dl) 1306 APTT () 28.4 IA (m/dl) 179 Fb (m/dl) 614 IM (m/dl) 143 D-dm (m/ml) 3.3 C3 (m/dl) 171 T-B (m/dl) 1.0 C4 (m/dl) 37.8 AST (IU/L) 15 CH50 (U/mL) 67.7 ALT (IU/L) 17 An-nu nbdy (d) < 79 LDH (IU/L) 192 PR3-ANCA (< 3.5 U/mL) 358 ALP (IU/L) 405 MPO-ANCA (< 9U/mL) 0.1 WBC, wh bd ; RBC, d bd ; PLT, p; APTT, vd p hmbpn m; Fb, bnn; FDP, bn ddn pdu; T-B, bubn; AST, p mnn; ALT, nn mnn; LDH, dhydn; ALP, kn phph; γ-gtp, γ-umy-nppd; ChE, hn; AMY, my; TP, pn; Ab, bumn; BUN, bd u nn; C, nn; SAA, um myd pn A; CRP, C-v pn; I, mmunbun; CH50, 50% hmy mpmn vy; ANCA, n-nuph ypm nbd; PR3, pn3; MPO, mypxd. nd hn w bvd. Lby xmnn vd nm uny, vn kn phph, um myd pn A, C-v pn, bnn, D- dm, nd mpmn vy. An-HTLV-1 nbdy w nv. An-nu nbdy nd mypxd (MPO)-ANCA w nv, bu h v PR3-ANCA, whh w xmnd by dn by hn h y, w hh (Tb 1). Ch X-y hwd nh bnm hdw n pu un. Cmpud mphy vd u m h b, pn nu, nd p, mxy nu, nd dun h mxy nu w (F. 1). Mn nn mn (MRI) vd pp nd wn h w b. Th n w w n T1-whd m, hnuy w n T2-whd m, nd pv dnum nhnmn, un nmmy hn h hn np (F. 1). Th w nmmn n h b. Pn mn mphy (PET) vd upk n h b, phynx, nd p, wh w d n h ubunu u (d n hwn). Ahuh h w n nvvmn h un nd kdny, h wh b nd n ympm, hh PR3-ANCA, h ndn, nd mn u, w ud h h pn ud m md yp WG. H umu n, hwv, pdy bm nd. A h n bpy hwd nh munud n n vu, whh yp h ndn n WG, bpy w pmd n m h hd m bw h yd. H w hn d wh pud mhypdnn. A mn, h pn pp, duy wwn, nd hn hwd mpvmn. Th hd m bw h yd d nd v u nmmy pn, uh SAA nd CRP, dd. H xmnn nd bpy vd nn m mdum-zd np ymphd, quny wh nm bnm m u, dmxd wh bundn v nmmy (F. 2). Th np hwd dv pn-t nn xpn, uh CD2 +/-,CD3 +,CD5 +/-,CD7 - nd CD4 +,CD8 -. CD10, CD20, CD79, CD56, EBER, nzym B nd TIA 1 w nv. K-67 bn ndx w 50%. Suhn b ny TCRβ n nmn w pmd; hwv, h nd bnd w n dd. W pu h h my b bu h v, n ymphm w dmnn n h bpy mp. Addn pym hn n ny dn TCRβ n nmn w n dn. W hn md dn dn pph T ymphm, n hw pd (WHO), nd h nvnn CHOP mn w d. W mud -ANCA by nd mmunun (IIF) dun CHOP mn whn PR3- ANCA w 74.7 U/mL, nd h u w nv. Th p- 2042

3 b d F u 1. CT ndmri nd n.s um h b ( ) ndp ( b)w d dbyct.onmri, h n n y h um h b w h n u y w nt2 w h d m ( ), nd w nh n dby d n um ( d). n nw n mp mn wh h v PR3ANCA dud 14.9 U/mL. Dun A n, WG uuy zd d wh numu n my n h upp py, whh hn pd ym vu. Ou mnn pd u n 28% 58% pn wh WG nd my b h n ympm n 8% 16% (3). Vu u mnn hv bn pd; vn my u n mny 8% pn (4). Ahuh m u ndn nnp, pp, whh w bvd n h pn, p pn nd, wh bu h pn wh pp pd vn du p nv hm (5). F dn b d, mpn dn bwn npm, nu, nd nmmy d. Cn MRI hpu n xmnn dn. In WG, dpndn n h h nmmy p, numu u n MRI n b dpd n dn n nny. In h n nmmy p, WG xhb hh-n nny n n T2-whd m. In h p numu nmn, num n b dmnd wn nny n n T2-whd m n WG. M nd ymphm pp - hypnn n T2-whd qun (6). MRI n h pn hwd hnuy w n nny n T2-whd m, whh ud WG h hn ymphm mnny. In ddn h ndn MRI, hh PR3ANCA w bvd n h pn. ANCA dd n nzn munph nd WG. Th mj nn nzd by ANCA n WG PR3. In ddn WG, PR3-ANCA h bn dd n pn wh ybunm vu, uv, nu d (ubu b ndd, ubu, py), n-ndud mdn duv n, humn mmundny vu nn wh n u, nd ngbm nbdy yndm (7-9). Hwv, unn whh h pn w ndd pv ypm ANCA (-ANCA), bu ANCA w dd by ELISA n h ud nd n by nd IIF un hn-xd nuph. Th xn vu nd mnn ymphm unmmn. Hwv, vu uh unu vu w pd b n n ympm mnn ymphm n m (10). Hmdu pd w ym -ANCA vu n h n hn ymphy ukm nd nmmunb T ymphm (11). In h pn h nd bpy m pb n vd nh numu n n vu, h nmmy hn my hv bn ud by T ymphm. T d, w PR3-ANCA-pv mnn ymphm whu vu hv bn pd. Zukmn

4 b d Fu2. Hndnndbpy.()Innnpymphd.(b) Bkwndm.()Immunhhmyvdhymphdw pvcd3nd(d)bncd7. pd pn wh nu, pu un, nd hh v PR3- nd MPO-ANCA, wh w dnd hvn hh-d nn-hdkn ymphm (NHL) (12). L pd pn wh pumny nn nd mdn ymph nd nmn nd hh v PR3- nd MPO-ANCA wh w dnd hvn wd B NHL (13). In h w, -ANCA w nv nd bh PR3- nd MPO-ANCA w ny pv. Th, n h h nbdy py nzd PR3. L ppd h B ymphm wh pmyd dnn mmunbun wh hh nnp ny h p n w ud n ELISA, un pvy PR3-ANCA nd MPO-ANCA. In h pn, h PR3-ANCA w hh h pv v. In ddn, h u w dn ELISA ndpndny pmd b nd dmn u hp w ny pv. Th bvn u h xn n PR3-nbdy, whh qu PR3-ANCA, n h pn um. On h h hnd, n ph ndn WG w bvd, un h pn hd PR3- ANCA whh w n d wh WG. Thuh h x mhnm ANCA pdun h n bn pvn, h wn md ppd. Inmmy ykn pdud by vu mu n h xpn PR3 n nuph. PR3 d m nuph pnd by nn pnn hp T. I uy T nhb h ummun pn, PR3- mud hp T n B nd nhn h pdun ANCA (14). In h pn, n PR3- nbdy mh b pdud by nmmy ykn nd mmun dyun ud by ymphm. Th mn vu w hu ky n h pn, bu u bvn ny. In nun, pv h hh PR3- ANCA, mpn dn bwn WG nd h d, uh nn nd mnny, nudn ymphm. Aknwdmn Th uh hnk h h Dpmn Hmy nd Rhumy, Thku Unvy hpu dun. Rn 1. Rnbum JT. Th y nd hum d. In: Ky Txbk Rhumy. 8h d. Fn GS, Budd RC, H ED J, MInn IB, Ruddy S, Sn JS, Ed. Sund, Phdph, 2009: G WL, Hn EC. Wn num: n nd mmundn p. In: Vu. 2nd d. B GV, Bd SL J, Ed. Oxd Unvy P, Nw Yk, 2008: Pku N, Sv D, Lbvh I. Wn num: ph- 2044

5 hm mnn nd mnmn. Smn Ah Rhum 35: , Bw J, Bbu K, Gp L, Khnkum S, Suh S, Rmkhnn S. Ou mnn Wn num. Any nn. Indn J Ophhm 51: , Cb LH, My ES, Dun G. Annuph ypm nbdy-d vu. In: Ky Txbk Rhumy. 8h d. Fn GS, Budd RC, H ED J, MInn IB, Ruddy S, Sn JS, Ed. Sund, Phdph, 2009: Muh C, Rnhd-K E, Rh C,. MRI h n vy, h pn nu nd b n Wn num. Eu Rd 7: , Wk A. Aunbd n vu. In: Vu. 2nd d. B GV, Bd SL J, Ed. Oxd Unvy P, Nw Yk, 2008: Wn O, Ru KA, L AS,. Annuph ypm nbd n wh humn nuph dn mk n-ndud mdn duv n bu n ummun vu. Ah Rhum 50: , Mnd LA, Smn DH, Smh EL, Lw RA, Kz JN, Shmn RH. Un nnuph ypm nbdy n dn vu: n -dn udn mpv dn uy? Ah Inn Md 162: , Smn Z, T T, Tóh L, Szu G. Cunu vu n nn pnp ympm n Hdkn ymphm. Rhum In 28: , Hmdu MA, E Ku D, Audn M, Gu JY. Sym nnuph ypm nbdy vu d wh ymphd np. Ann Rhum D 60: , Zukmn KK, Lvnh L, Wynn C. Pv -ANCA n Pn wh Lymphm nd Whu Vu. Cn Rhum 3: , L AS, Wn O, Gp DJ, Wz TE, Hmbu H, Spk U. A 70-y-d mn wh pumny n nd pv nnuph ypm unbdy u. Ch 127: , Abduhd WH, Smn CA, Knb CG. Rvw : Th CD4 (+) T n ANCA-d ym vu. Nphy 14: 26-32, Th Jpn Sy Inn Mdn hp:// 2045