TAP CHl V - Diroc HOC OUflN SU S6' 8-2014
NGHIEN ciru OAC DIEM LAIVI SANG, CAN LAM SANG U LYMPHO AC TfNH NON-HODGKIN NGUYEN PHAT NGOAI HACH TAI
BENH VIEN QUAN Y 103 GIAI DOAN 2008 - 2014
Nghiem Thj Minh Chau' TOM T A T
NghiSn ciru hoi cdu 30 blnh nhan (BN) u lympho ac tinh non-Hodgkin (ULPNH) nguySn phat ngoSi hach tai BSnh viSn Quan y 103 giai dogn 2008 - 2014, tac gii rCit ra mOt s l kit lujn;
U lympho ac tinh nguySn phat ngoSi hach chllm 4,4% ting s l BN ULPNH va xuat hiSn 6 nhilu vi tri, eg quan nhu: dudng tieu hda: 56,67%, mit: 13,33%, da: 10%. T^ l | nam/nO. 2,33/1.
Bd tuli tmng blnh: 46,6 ± 17,36
Sit la triSu chirng gap phi biln va 63,33% BN cd It nhat 1 triSu chyng "B".
30% BN tang nong dd LDH mau.
90% BN tin thucng t l bao B va 83,33% cd grad trung blnh.
76,67% cd chi s6 IPI tot tgl thdi dilm vao viSn.
* TU' khda: U lympho 4c tinh non-Hodgkin ngoai hgch nguySn phat; Ble dilm ISm sSng, cjn ISm sang.
RESEARCH ON CLINICAL, SUBCLINICAL CHARACTERISTICS OF PRIMARY EXTRANODAL NON-HODGKIN LYMPHOMA
AT 103 HOSPITAL (2008 - 2014)
SUMMARY
Ttte descriptive-analytic study was carried out on 30 patients, wtio were diagnosed tt\e pnmary extranodal non-l-iodgf<in lymphoma (PENHL) at 103 Hospital from 2008 to 2014. ths results siiowed tt^at:
- PENHL accounted for 24.4% NHL and was distributed in many sites/organs suci) as gastnyintestinal tract (56.67%), eyes (13.33%) and derma (10.00%). Tt)e ratio of male/female was 2.33/1. Mean age: 45.5 ± 17.35 years old.
- Fever is ttie most common symptom and 63.33% of patients had at least 1 symptom "8"
- 30% of patients increased blood levels of LDH.
- 90% of patients iiad B cell damage and 83.33% had an average grad.
- 76.67% had an IPI good index at the time of admission.
' Key wads: Primary extranodal non-Hodgkin lymphoma; Clinical and paraclinical characteristics.
* B$nh vi$n QuSn y 103
Ngu^phin h6i (Corresponding): Nghiem Thj Minh ChSu ([email protected]) Ngdy nhSn bhi: 25/07/2014; Ng^y phdn bi§n danh gi6 bdi bao: IS/09/2014
Ngdy bdi bao tfuwc ddng: 25/09/2014
TAP CHl V - Diroc HOC OURN SU SO 8-2014 B A T VAN BE
U lympho I c tinh khlng Hodgkin I I m|t trong 10 bgnh ung thy thydng g l p g ngydi Ign. Bgnh cd bilu hien l l m sing da dgng v l cd xu hydng lan trin. T i n thygng eg b i n ciia b|nh I I tgl hgch lympho, tuy nhiln cd t h i cd t i n thygng ngoii hgch p h l i hgp. U lympho ac tinh nguyen phat ngoii hgch 11 ULPNH cd hinh I n h t l bao lympho benh ly nguyin phat tgl mgt vj tri/cg quan ngoai hgch lymphp, ed t h i cd hole khdng kem theo t i n thygng hgch vung giai dogn le; HE [3].
Ty l | ULPNH nguyin phit ngcai hgch gap tCr 20 - 40% tong s l ULPNH [1, 2], Bilu hign ban d i u eua b|nh xuit hiln g nhilu eg quan khIe nhau, tuy thule vj tri nguyen phit, dg v|y, BN ed t h i d i n khim g ele chuyin khoa khic trydc khi dygc dilu trj dgc hi|u diing chuyen nglnh.
flI ed kiln thirc chung va djnh hydng ehin deln chinh xac hgn d l i vdi ULPNH nguyin phit ngoii hgch, chiing t l i tiln hlnh a l tai n i y nhim: Xic djnh nhOvg die diim lam sing vi cin lim sing eua BN ULPNH nguyin phit ngoii h$ch.
BOI TUONG VA PHUONG PHAP NGHI£N Ciru 1. B i i tu'gng nghien CCFU.
BN dyge ehin doin x i c djnh I I ULPNH ngpli hgch dilu trj tgi Bgnh vien Quin y 103, khlng phin bi|t t u l i , gidi.
* Tieu chuin chpn:
T i t ea BN ULPNH nguyin phit ngeli hgch trong s l nhO-ng BN ULPNH dilu trj tgi B|nh vi|n Q u i n y 103 tO' 01 - 2008 d i n 01 - 2014 dyge chin deln x i c djnh bang giai p h i u b l n h ly hcgc hoa m l miln dich.
* Tiiu chuin ioai trir: BN d i n g gigi dogn lan tran, khdng x i c djnh dyge t i n
thygng nguyen phit chic ehan I I tai hgch hgy ngcli hgch; BN khlng hgp tie;
BN khlng x I p Ipgi dygc thee bang phin Ipai eua Werking Formulation (WF).
2. Phu'ang p h i p nghien cirU.
Theo doi dgc k i t hgp m l ta h l i cu'u.
* Cac tieu ehuin ip dpng trong nghien eiru: bang phan logi Working Formulgtion- WF. Tnj'dng hgp k i t qua m l blnh hgc ehi mo ta hinh anh m l khlng x I p logi, s l ein eCru vio hinh anh md ta d l phien sang bang phin loai WF; chi s l t i l n lygng q u i o te d l i vdi ULPNH: Internatidnal Progngsticlndex-IPI.
' Xir if so iiiu: b i n g p h i n mem Epi.infc 6.0.
KET QUA NGHIEN CLfU VA BAN LUAN
1. Bae d i l m v l t u l i va gid'i cua nhdm nghien cipu.
Nam: 21 BN (70,00%); nu': 9 BN (30,00%); t u l i trung binh (X ± SD):
45,5 ±17,35.
Trong nghien cCru, nam ehilm ty l | cao hgn nu'. Kit qua n i y phii hgp vdi m|t so nghiin ciru trong v l ngoii nyge [1, 2, 5],
2. Ty l | ULPNH nguyen phat ngoai hach cua n g h i i n cCru so v g i m^t s i t i c gia khac.
Bing 1:
TAC GiA Krai A.D.G (2003) Gurney K.A (2002) L6 T ^ n £)9t, Phgm Xudn DEJng v^ CS (2005) Chiing I6i (2014)
T6NG ULPNH 11S4 11 334
524
124
ULPNH N G U Y S N PHAT NGOAl HACH
389 3356 142
30 T f L f
{%) 34 31,4 27,1
24,2
107
THP CHi V - DUOC HOC QUfiN Slf S6" 8-2014 Ty le ULPNH nguyin phat ngoii hgch trong nghiin ciru n i y tygng t y kit qua ciia L l T i n Bat, Phgm Xuin Dung v l CS (2005) [2], tuy nhiln, ty l | eCla chung t l i thip hgn cua m§t s l t i c gia nydc ngoii [4, 6, 7). Bilu niy cd the do m|t s l BN ULPNH cua chiing tdi d i n blnh vi|n khi da g giai dggn lan trin, khd xic djnh t i n thygng nguyin phit I I tgi hach hay ngeli hgch, dc v|y, nhu'ng tnj'dng hgp niy khdng dyge chgn v i o m l u nghiin ciru. Theo Asenberg A.C (1991) [4], Doll D.C (1999) [6], u lumpho l e tinh nguyin phit ngoai hgch 11 ULPNH ed hinh anh t l b l e lympho b|nh \^ nguyin phit tgi m|t vi tri/cg quan ngcai hgch lympho, cd t h i cd hogo khdng k i m theo t i n thygng hgch vung - giai dogn IE; ME. M|t khic, phan b l v l mgt dia dy sdng v l chOng t|c, mdt s l t i c gia nydc nggai nh|n thiy ULPNH the mit gap nhilu hgn g chau Phi v l ngydi da m l u [8]. Nhy v|y, sy khic nhau n i y cung cd t h i do ehung tie.
3. P h i n b i theo vj t r i ton t h u g n g nguyen p h i t .
Sang 2:
Do vj tri t i n thygng nguyin phit rit da dgng, BN c l t h i vao cae khea khic nhau tnj'dc khi dygc chuyin d i n dung chuyin khea. Trcng nghiin cCru nay, 56,67%
ULPNH nguyen phat ngoai hach bilu hi|n tgi dygng tieu hda, cao hgn m|t so tac gia da edng bo. Nghiin cCru cua Sutelifee S.B, Gosppdarevicz M.K (1998) la 24,3%, cua Le T i n Bgt, Phgm X u i n Dung v l CS (2005) [2] I I 14,1%, cOa Nguyin Ba Birc va CS (1995) la 10%. Nhy v|y, ele kit qua r i t k h i c nhau. Tuy nhien, nhii'ng nghiin eiru tren d l u tien hlnh tCr nlm 2005 trg v l trydc, khi m l kJ thugt nhulm hda m l miln djch d nydc ta chya ph6 biln, do v | y cd t h i m|t s l trydng hgp u g dg dly, rudt bj bo sdt, khlng dygc chin doin x i c djnh I I ULPNH. T i n thygng nguyen phit tgl cae vj tri khac trong nghiin eCru cua ehung t l i ehilm ty l | thip, nhyng do mau nghiin eCru n i y cdn it, moi vi tri ton thygng chi gap 1 tm'dng hgp n i n khlng b i n lu|n v i n d l niy m l chi dya ra s l lilu d l tham khae.
4. Tri|u chti'ng l l m s i n g khi v i o vi|n.
Bing 3:
VjTRl
ti&u h6a D a d d y RuOt Mac treo M i l
Da Tinh ho^n NSo Ph6i Xu'ang Vii Tuyen (re
Sd LUONG
6 9 2 4 3
Tin
20,00 30,00 6,67
56,67%
13,33 10,00 3,33 3,33 3,33 3,33 3,33 3,33
TRltU eHiING
Tri$u chOng"B"
Sot Gdy sUt c3n Ra m l hoi C6 it nhat 1 triSu chirng B Hach vCing
TriSu chirng khSc tai co quan ton thoong (ngodi triSu ChCrng c6 khoi u)
S6 LLTONG
19 11 12 19 8 30
Ti-Le
63,33 38,67 40,00 63,33 26,87 100
Triiu chirng l l m s i n g eua BN eung da dang v l phy thupc vao eg quan t i n thygng nguyen phit. 63,33% BN ed it nhit 1 trigu ehirng "B" v l triiu chirng gap
T9P CHi V - Diroc HOC 0 U 8 N SU SO 8-2014 nhilu nhit I I s i t . S i t eua b|nh hach I c
tinh CO d i e dilm rieng khac vdi triiu ChCrng s i t trong mgt s l blnh 1^ khic. Dc v|y, nam vu'ng d i e dilm trieu chirng s i t ed y nghTa trong djnh hydng ehin doin ban d i u . CIc triiu chirng g i y silt e i n v l ra mo h l i gap vgi ty I I t h i p hgn. Trieu chirng tgi c i c eg qugn t i n thygng nguyin phit cd t h i la: r l i lean tieu hda, nhin md, nhCre h i e mit, ngu'a ngoai da. .
5. T r i | u chCi'ng cgn lam sang khi v i o v i | n .
Giam huylt s i c to: 4 BN (13,33%);
LDH/mau tang: 9 BN (30,00%).
Trong nhdm nghien ciru, 4 BN eo bilu hi|n thilu m l u mii'c dd nhg - trung binh, dlu I I BN ed t i n thygng nguyen phit tgi dygng tilu hda. NhO'ng BN n i y di ngoii ra mlu va thilu mau la do h|u qua mit mlu man tinh, khao s i t tuy d l cua BN chya thiy tinh trgng lan trin tuy xygng.
LDH/mlu I BN ULPNH I I mpt trong nhu'ng ylu t l tinh dilm cho ehi s l t i l n lygng IPI.
Chung tdi thay 30% BN cd ehi s l LDH/mlu ting tgi thdi diem vao vi|n, c l o BN deu giam ehi s l LDH sau 3 - 6 chu ky dilu trj.
Kit qua n i y phii hgp vdi nghiin ciru eua L l T i n Bgt v l CS (2005) [2]: 47,5% BN u lympho I c tinh nguyen phat ngpli hgch ed ting LDH/mlu. Tuy v|y, Pham Xuan Dung v l CS (2003) nghiin cu'u ehi s l n i y trin BN ULPNH ngydi ldn thiy ehi s l LDH/mlu tang g 65,1%p.
LDH cd trong b i o tygng ciia h i u h i t clo mo. Hgch la t l chCpc du'ng thu' 5 trong s l 10 cg quan giau LDH nhat eg t h i . Chi s l LDH mau toan phin v l ty Ig isgenzym cua LDH dygc diing nhy m|t ehi tieu thee del tinh trgng hogt dgng cua khli u lien quan vgi eg quan tgo mau [10].
Trong nghiin cyu niy, chiing tdi v i n ap dyng x I p Iggi gill phiu benh theo eIng thCrc thyc hlnh eua IVIy (Working Fprmulatipn-WF), do dilu ki|n x i t nghiem eiia b|nh vi|n. Cho d i n nay, nhilu nghiin CU'U treng v l ngeli nydc da nh|n thiy xIp loai WF cdn nhilu han c h l , nhit I I del vdl u lympho ac tinh nguyin phit ngoii hgch. IVIgt s l t h i b|nh eua u lympho l e tinh nguyin p h i t ngoii hach chya hoge khlng t h i x I p loai theo phin leal n i y . H i l n nay, bang p h i n logi REAL (Revised European - American Lymphoma Classifieation) d y g c sir dung rdng rai hgn dg da d l elp d i n ele t h i MALT, t h i t l b i g T hole NK, I I nhu'ng t h i thydng ehi ed g u lympho I c tinh nguyin phat ngoii hach, nhyng each phan Ipai nay chya dygc ap dung tai B|nh vi|n Quin y 103 v l nhilu blnh vi|n khic do dieu kien xet nghiem.
6. Phan b l theo phan dd md hoc (WF).
Bang 4:
PHAN DO MO HOC
Grad thlp
Grad trung blnh (vCra)
Grad cao
WF1 WF2 WF3 WF4 WF5 WF6 WF7 WF8 WF9 WF10
S6
LUONG ri L|(%)
3 3 16 7 8
10 10 63,00 23,33 26,67
83,33
26,67
Ty le BN cd grad trung binh ehilm cao nhit (83,33%). Kit qua ciia chiing toi tygng dygng vdi nghien ciru eua B|nh vien K 109
T f P CHi V - DUOC HOC QURN SU SO 8-2014
H I Ndi giai doan 1983 - 1993, nhyng thip hgn k i t qua eiia Vi|n Ung thy Quoc gia IMy (1982). B i l u nay ed the do sy khic bi|t v l ehCing tgc.
7. Phan bd theg loai t l bao va t h i benh.
Bing 5:
S6 lu'gng Ty 1* (%)
LOAI Jt B A O Te bao
B 27 90,00
T i b a o T 3 10,00
THE B E N H The Ian toa
28 93,33
Th4 nang 2 6,67 Trong s l ele BN nghiin cCru, chung tdi phat hien 3 trydng hgp t h i t l b i o T, deu 11 BN ULPNH nguyen phat g da. Nam 2010, x i t nghiim hda md miln dich mdi dygc chi djnh ring rai tai Benh vi|n Quin y 103. Do vly, cd t h i s l BN cd the t l b i o T, MALT hole te b i o NK bj bo sdt trong nhu'ng thdi gian tm'gc. Tnj'dc d l y khi d l c|p d i n u lympho da nguyen phat, ngydi ta chi ndi d i n u lympho da nguyen phat dgng t l ble T. Mac du hien nay, lympho da nguyin phat dgng t l b l e B d l dygc xic djnh, nhyng nd ehilm ty I I thip hgn nhilu so vdi dang t l b i g T. U lympho ae tinh nguyin phat d da hien dygc x I p loai theo 2 bang phin logi: European Organization for Research and Treatment of Cancer (EORTC) v l WHO. Vigc xac djnh typ t l b i o r l t e i n thilt, vi nd Iiln quan d i n phle do a l u trj. Treng khi u lympho da nguyen phit te b i o B dieu trj nhy ULPNH ndi chung, u lymphd da nguyin phit t l b i o T lya chpn dilu trj tygng Crng vdi giai dean theo NCI - My la:
lieu phip anh s i n g vdi tia eye tim A vl/holc xa tri chCim tia electron toan bg da k i t hgp bdi mustart tgi c h l vl/hoac
hda trj tpIn t h i n k i t hgp... Trgng nghiin eyu niy, chya cd BN nag dilu trj ligu phip anh s i n g vdi tia eye tim A vl/hp|c xa trj chCim tia electron t o i n b | da kit hgp boi mustart tgi c h l .
8. Banh gia t i l n lu'g'ng thd'i d i l m BN v i o v i | n ( d ^ tren chi s l t i l n lu'gng IPI).
Tiln lygng t i t (dilm IPI 0 - 1): 23 BN (76,67%); tien lygng trung binh (IPI 2 - 3):
7 BN (23,33%); tien lygng x i u (IPI 4 - 6):
OBN.
Treng s l 30 BN nghien eiru, khdng BN nio cd chi s l t i l n lygng (IPI) xlu, 76,67%i tien lygng tit. K i t qua ciia chiing tdi tygng t y nghien eiru cda Le T i n Dal v l CS (2005) [2]. Nhy vgy, n l u lya chon phic d l phu hgp, BN se ed eg h|i klo dai thdi gian song t o i n bp v l thdi gian song thlm b|nh khlng tiln triln.
Kg'T L U A N
Qua nghien eCru dgc diem l l m sing v l e|n l l m sing bgnh ULPNH nguyin phit ngoai hgch tgi B|nh vi|n Quan y 103 giai dean 2008 - 2014, chOng toi riit ra kit lu|n:
- U lympho l e tinh nguyen phit ngoai hgch ehilm 4,4%) t i n g s l BN ULPNH.
Nam ehilm ty Ig eao hgn nCr. B l tuli trung binh 45,5 ± 17,35 t u l i .
- Ton thygng nguyen phat g dydng t i l u hda ehilm ty I | eao nhit (56,67%), sau dd I I mat (13,33%) v l da (10,00%).
- S i t I I trigu chirng gap p h i biln v l 63,33% BN ed it nhit 1 tri|u ehirng "B".
- LDH m l u tang g 30% BN.
- 90%) BN t i n thygng t l b i g B va 83,33%) CO grad trung binh.
- 76,67% Cd chi s l IPI t i t tgi thdi dilm
T9P CHi V - Diroc Hpc O U A N S I / sd' 8-2014
T A I L I E U T H A M K H A O 1. Ph^m HOng Cu^ng. Lympho nguyen phdt dirfrng tiSu hoa: C h i n doSn vd dieu tri.
Tgp chl Y hpc TP H 6 Chl Minh 2001, phu ban s64, t 0 p 4 . tr.49-51.
2. LS Tin Bat, Pham XuSn DDng, NguySn Hdng Hii vd CS. U lympho khdng Hodgkin ngodi hgch nguy&n pheit ngu'd'i 16'n: C h i n dodn vd di4u tri. Tgp chl Y hpc TP. H 6 Chl Minh.
2005, phy bdn s6 4, tap 9, tr.585- 595.
3. Luv HCing Vu. Ph$m XuSn DOng. Bieu trj lympho da nguySn phdt tglBgnh vi0n Ung bu-du TP H 6 Chl Minh: Bdo cdo 4 tucej-ng h(?p Tgp chi Y hpc. TP. H 6 Chl Minh. 2005, phy bdn so 4, tdp 9,tr.l
4 Aisenberg A.C. Extralnodal lymphoma:
Presentation natural history and special treatment consideration in Aisenberg A.C.
Malignantnlymphoma; Biology, natural histology anad treatment. Lea & Febiger, Philadelphia- London, USA. 1991, pp.235-310.
5. Connors M.J, His D.E, Foss M. F.
Lymphoma of the skin. The American Society of Hematology. 2002.
6- Doll D. C. I n t r o d u c t i o n : Extranodal lymphomas, Semin Oncol. 1999, 26 (3), pp.
249-250.
7. Krol A.D.G, Le Cessie S, Snijder S et al.
Primary extranodal non-Hodgkin lymphoma, impact of alternative definitions tested In comprehensive Centre West population-based ULPNH registry Ann Oncol. 2003,14, pp. 31-139.
8 Gurney K A, CartwrigthRA Increasing incidence and descriptive epidemiology of extranodal non- Hodgkkin's lymphoma in parts of England and Wales, The Hematol J 2002, 3, pp.95-104,
9. Pandolfino L. T et ai. Primary cutaneous B-cell lymphoma: Review and current concepts Journal of Clinical Oncology, 2000, Vol 18, issue 10 (May), pp.2152-2168.
10. Robert S. Hillman, Kenneth A.Ault, Michel Leporrier et al Non-Hodgkin Lymphomas 2011, pp.279-300.
