CHUYBN Bt: HOI NOHI KHOA HQC HU'
13. Tefferi A., Varian JW. (2008),
"Classification and diagnosis of myeloproliferative neoplasms: the 2008 World Health Organization criteria and point-of care diagnostic algorithms", Leukemla.22,pp. \4-22.
14. Tefferi Ayalew, Shireen SIrhan, Terra L.
Lasho (2005), "Concomitant neutrophil J/UC2V6I7F mutation screening and PRV-I
fS^ i • •- - iV^>UYiM|lAU ToAW QUgC 2012
^txprauion analysis ui myaloptolifi diaotden and secondary polyi British Journal of Haematology, 131, pp, 166-177. i^
IS. Xiao Z., ^hang Y., Li L. (2008), "Hie Janus Kinlse 2 (JAK2) V6I7F rautatioa In Chinese patients with chronic myeloproliferative disorders', Haematologica 93, pp. 787-788.
DAC DI^M TdN THITDNG TIM MACH
CUA BlNH NHAN THALASSEMIA Vii NANG TAI BlNH VICN NHI B(iNG I
T6M T&T
Mpc tiiu: Mi t i cic die diem tSn ttiudng bm mach cua binh nhin thalassemia thi ning nhap viin diiu tt| tpi bpnh vipn Nhl Ddng 1 t i tiling 2 din thing 8 nim 2011.
PhU'dng phip: Tien ciu mi t i hing loat ca bin 62 binh nhin nh$p viin Nhi CiSng 1 t i tiling 2 den tiling 8 nim 2011. TSt d cic binh nhan aiKfc hdi binh si, khim lam sing vi thpt:
hiin cic xet nghiim nhu cdng tiiuC miu, ferritin, X quang ngpc thing, do ECG va siiu im bm Doppler miu.
Ket qui: Ti li ton thuUng tim mpch li 83,9% bong dd dan budng tim chiem 77,4%, ting ip phil chiem 24,2%, phi dpi thit bii chiem 17,7%, suy tim chiem 12,9%. Cic lopl tin thudng cd Bii chong Ian len nhau, suy tim va
• Bg mdn Nhl Dal hgc Y Dupe TP.HCM, Phan bipn khoa hpc: TS. Huynh NghTa
D i o Thi Thanh An*, L£m Thj M;'
ting ip ph6l thuOng xuit hiin dong ttidl. Binh nhin tdn ttiutmg dm mpch khdi phit binh sdm vi cin truyin miu sdm hdn nhdm khing t&i thuong tim mpch. Nhdm tSn thudng tim cd ti ii ttiieu miu v i i sit cao hdn nhUhg ti li dupe thii sit ipi thip hdn.
Kit lu$n: Ti l | ton thuong tim manh bin binh nhin thalassemia tttS ning rat cao, cic biin ching tim mpch r€t da dpng vi cd thi xuSt hlin cing lie.
ABSTRACT
HEART COMPLICATIONS I N PAHENTS WITH THALASSEMIA MA30R AT CHILDREN'S HOSPITAL NUMBER 1 Objective: described the heart oomplkaBons In patients witii thalassemia major at
70
Y HQC VlgT NAM THANG 8 - SQ' BAC BlgT/ZOI 2 Children's hospital number 1 at Ho Chi Minh City.
Methods: We described heart complications in 62 thalassemia major patients at Children's Hospital Noi from February 2011 to August 2011 (age under 15 years old). All patients were taken medical history, physical examination, checked complete blood count, ferritin, ECG, chest X-ray, and Doppler Echocardiography,
Results: Heart complication was recorded in 83,9%, including 77,4% heart dilation, 24,2%
pulmonary hypertension, 17,7% left ventricular hypertrophy, 12,9% congestive heart failure.
Heart failure and pulmonary hypertension were coewsting. Patients with heart complication were younger, started earlier and transfused earlier than patients without heart complication. Heart complication patients had severe anemia and iron overload than other groups however there was smaller rate of chealation and transfusion In this group.
Conclusion: the proportion of heart complication in patients with thalassemia major was high. There were various types of complication however they a}uld appear at the same time.
I. DAI CaONG
Thalassemia la mot trong nhihig b$nh ly di truyin thu6ng g$p nhit trgn th€ gidri.
Benh nhSn thalassemia the n$ng c6 tinh trang thiiu mau n ^ g khdi phat som can truyin mau su6t doi dl duy tri su s6ng vk phdt triln,
niu khdng dugrc diiu tri sS dUn din nhfhig biin chCmg \k h$u qu^ ci^a tinh tr^g thiiu mdu t6n huyit mSn vk tinh tr^ng thiiu oxy md cdng nhu cdc phdn Omg bCi trit cit& ca thi.
Lugng sit sS tfch ty 6 gan, phii d$c bi^t 6 tim hlnh thdnh nSn cdc glc t^r do gSy hxiy ho^ii cdc tl bdo vd htiy ho^i s\r. ting hpp collagen ciia ca thi. B§nh tim mgch Id nguySn nhdn hdng dAu gdy ti!r vong trSn b$nh nhfin thalassemia, trong d6 bao gIm biin chumg chO yiu Id suy tim, tdng dp phii, rii lo^n chiJTC nfing tfim thu, rii lo^n chiic ndng tdm truong thit trdi vd rii l o ^ nhip tim. Vi$t Nam c6 sl lu;i;mg b$nh nhdn thalassemia rit cao, hing ndm b^nh vi^n Nhi Ding 1 tiip n h ^ mOt lugng 16n b$nh nhan thalassemia the n$ng vdo diiu tri truyin mdu vd thdi sdt.
Tuy nhien bien chung tim mach trong thalassemia thi ndng vSn chua dugrc khdo sdt diy di^t. D6 Id ly do chung toi th\rc hi^n nghien cijru nay.
II. PHUONG PHAP NGHIEN OJTU
Thilt kl nghien cuu: tiln cvru md ta hdng lo^t ca.
Ddn sl myc tieu: Tat cd cdc tre dual 15 tuoi dugrc chin dodn thalassemia the n|ng.
Dan s6 chQn mSu: Tat cd cac trg dugrc chin dodn thalassemia thi n$ng nh$p Khoa Huyit hgc bgnh vi?n Nhi D5ng 1 tir thdng 2 din thdng 8 ndm 2011 dong y tham gia nghien cuu.
III. KET QuA
Xac dinh ti I? tin thmrag tim mach: Ti 1? tin thuorng tim mgch trong nghidn cihi Id 83,9% (52/62)
Bdng 1. Ti 1| cdc loai tin thuong tim mach:
1
C^c lo^i ton thirdng tim m^ch (N=621 Dan buong tim
Til$
(48/52) 77,4 %
PhSn lo^l
That phai That trai
Tilf (12/62) 19,4 %
(12/62)19,4%
CHUYBN Pg: HQI NOHI KHOA HQC HUYgl HQC • TRUYJN MAU TOAN QU6C 2012
2
3 4
5 6 7 8
Ting ip phSl
Phi dai ttiit trii Suy tim
Giim CO b6p CO tim R6i loan nhip tim Trin dich ming tim Suy chik ninq tim truanq thit trii
(15/62) 24,2%
(11/62) 17,7%
(8/62) 12,9%
(4/62) 6,5%
(2/62) 3,2%
(1/62) 1,6%
0%
Hai thit D61 W 2 0 6 3
(24/62)38,7%
(12/62) 19,3%
(2/62) 3,2%
(1/62) 1,6%
O i l 002 D«3 D04
(3/62) 4.8%
(3/62) 4,8%
(1/62) 1,6%
(1/62) 1,6%
Xic djnh ti 1$, so trung binh cic die diim ciia b^nh nhi (tu6i, gidi. thi b^nh, noi cu ngy, dinh duSng), ngufri nuAi duSng (Itinh ti, vin hda), cin lim sing, dieu trj ciia b^nh nhin thalassemia thi ning c6 tSn thuong tim m^ch vi kh6ng l6n thuong tim m^ch:
Bing 2. Die diim ciia b^nh nhin thalassemia thi ning c6 t6n tiiiKmg tim m ^ h vi Idiong tin thuong tim mgch
e$c diem b$nh nhSn thalassemia t h i ning
Tuoi benh nhan (tuoi) (TB ± OLC) Tuoi phat hien benh (tuoO (TB ± DLC) Tuoi truyen mau fan dau (tuoi) (TB ± DLC) Gidi nam (%)
Cu' nqu 6 tinh (%)
Suy dinh du8nq trunq binh, ninq (%) 13 thalassemia (%)
P thalassemia/ Hb E (%) 0 thalassemia (%)
Khdng t6n thytfng tim mach (n=10]
8,7 ± 1,1 2,7 ± 0,6 2,7 ± 0,6 6/10 (60%) 8/10 (80%) 5/10 (50%) 4/10 (40%) 5/10 (50%) 1/10 (10%)
C6 tSn thifong tim m^ch (n=52)
7.2 ± 0,4 1,7 ± 0,2 1,7 ± 0 , 2 . 26/52 (50%) 17/52 (71,2%) 31/52 (59,6%) 32/52 (62,5%)
16/52 (30,8%) 4/52 (7,7%)
Bang 3. Die diim ve hemoglobin, ferritin ciia b$nh nhan Uialassemia thi ning c6 ton thuong tim mach va jdions tin thuong tim mach.
Sic diim b^nh nhin thalassemia thg ning
Hemoglobin (q/di) (TB ± DLC^
< 6 q/dl 6-9 q/di
Khong ton thu'bng tim mach (n=10)
6,4 ± 0,2 1 / 1 0 ( 1 0 % )
9/lC (90%)
C6 tSn thu'tfng tim mjch (n=52)
6,1 ± 0,2 22/52 (42.3%)
30/52 (57,7%)
72
Y HQC VigT NAM T H A N O 8 - S 6 DJlC BI$Ta012
Femtln (nq/ml) (TB ± OLC)
< 1000 nq/ml (%) 1000-2500 nq/ml (%)
> 2500 nq/ml (%)
2277 ± 356,7 1/10 (10 %) 6/10 (60%) 3 / 1 0 (30%)
3040,5 ± 423,9 17/52 (32,7%) 14/52 (26,9%) 2 1 / 5 2 (40,4%) Bing 4. Die diim vi thi binh, diiu trj eiia b$nh nhin thalassemia thi ning c6 tin thucmg tim mach vi kh6ng tin thuong tim mach.
e$c.dilm bfnh nhin thalassemia th€ nitng S€ lin truySn miu trong nim (lin) (IB ± BLC)
<10 ian(%)
> 10 Ian (%) 03 thii sit (%) Ca d t lich (%)
Khdng t6n tiiiTtfng tim m^ch (n=10) 1 2 , 4 1 0,6
1/10 (10%) 9/10 (90%) 8 / 1 0 ( 8 0 % ) 1/10 (10%)
CA tdn thiMng tim m9ch (ns52)
10,3 ± 0,5
17/52 (32,7%) 35/52 (67,3%) 33/52 (63,5%)
11/52 (21,2%)
IV. BAN LU$N
Trong th6i gian nghi§n cihi chi!ing toi dd thu th$p dugic 62 b|nh nhan th6a tieu chi chin dodn.
Ti 1$ tin thinmg tim m^ch tren b^nh nhSn Thalassemia thi nSng
Co 83,9% (52/62) bfnh nhan c6 tin thuong tun m^ch v6i dinh nghia bao gom suy tim, tdng dp phii, phi d^i thit trdi, ddn that, gidm CO bop ca tim, trdn dich mdng tim, roi l o ^ nhip tim.
DSn buing tim
Trong nghien cihi ciia chiing t6i ti 1? ddn buong tim cao hon so nghien cuu ciia tdc gid IvB Phucmg Hanh ndm 1998 la 40,6%. Ti 1$ ddn thit trdi ^m thuin vd ddn thdt phai nhu nhau.
Biin chimg ting dp phii: Chiing toi ghi nhinti le ^ng dp phii Id 24^%.
Co chi chinh gdy tdng dp phoi Id do tinh trai^ thieu oxy mo mdn vd tdn huyet m£in tinh. B^nh nhan thi ndng ciia chiing toi khong duy tri dugrc mure hemoglobin mong muon nen phai chju hau qud cua tinh trang thiiu oxy man nhu nhihig b?nh nhdn thi trung gian.
Phi dQi thit trdi
Chiing t6i ghi nh$n dutyc 17,7% (11/62) b€nh nhdn cd phi dai that trdi. Trong nhOng b6nh nhdn ndy chi co 27,3% (3/11) bfnh nhdn phi dai thit trdi dom tiiuan trong khi 72,7%
(8/11) bfnh nhdn k^m theo ddn tiilt Irdi.
Biin chihig si^ tim
NghiSn cijru cua chiing t6i c6 ti If suy tim cao hon so v6i nghi€n ciiu cua Athanasios Aessopos va c^ng s\r vdo ndm 2003 tren 131 bfnh nhan thalassemia thi n$ng. Sir khdc blft Idn ve kit qud nay Id do dii tugng nghien ciiu ciia Athanasios Aessopos Id nhthig bfnh nhdn dugc truyen mdu thuong xuyen duy tri miic hemoglobin tren 9 g/dl trudc truyin mdu ding thai bfnh nhdn dugc thdi sit diu d$n rit sdm sau truyin mdu iln dau. Trong khi bfnh nhan ciia chiing t6i tat ca diu c6 miic hemoglobin dudi 9 g/dl trudc khi truyin mau vd thdi sdt kh6ng thudng xuySn.
Giant co bdp cat tim
NghiSn Cliu cua chung toi cd ti If benh nhan giam siic co bdp co tim la 6,5% (4/62).
Theo bdo cao ndm 2007 ciia Aessopos so
C H U Y S N efe H O I NOHI KHOA HQC HUYJtti <lllAUTOANQUtfC2012
sinh hai nhdm binh nhin thalassemia thi ning (n-131) vi thi trung gian (n-74) thi tinh b-?ng suy chiic ning tim thu thdt trii chi gip hung thalassemia thi nJng vcSri tl li 8%.
Rii lopn nhip lim
C6 1,6% (1/62) binh nhin block nhinh phii, 1,6% (1/62) binh nhin r6i lo?n nhjp nhl, hai binh nhin niy Ithftng c6 ttiiu chiimg suy tim vi chi phit hiin tinh c6 Ichi do ECG.
Trin djch ming tim
Kit qui c6 1,6 % (1/62) binh nhin trin djch ming tim b^n siiu im.
Chic ning tim Irmmg thit trii Ik ci b?nh nhin diu c6 ti li E/A < 1 chimg td 100% (62/62) bfnh nhin c6 chiic ning tim truong thAt trii bio tin.
Xic djnh ti \i, s6 trung binh cic die diim cOa binh nhi (tuii, gidri, thi bfnh, noi cu ngu, dinh duSng), ngudi nudi dudng (kinh ti, vin hda), cin lim sing, diiu tr) cua bfnh nhin thalassemia thi ning ed tin thuong tim m^h vi khdng ton thuong tim m^ch:
Bi^c diim cff d^a cua bfnh nhin thalassemia thi n^ng ci ton thuvng tim mpch vi khdng tin Ihutntg tim mpch
Nhdm binh nhan cd tdn thucmg tim m^ch cd d^ tuoi tnmg binh ii 7,2 ± 0,4 tuii, hoi thap hon nhdm khdng cd tin thuong tim m?eh li 8,7 ± 1,1 tuoi. Tuoi phit hi$n bfnh vi tuii truyin miu Ian dau ciia nhdm tin thuong tim m^eh sdm hon nhdm khdng tin thuong tim mjch. Ti if suy dinh dudng trong nhdm ton thuong tim m?ch cao hon nhdm khong tin thuong.
Bfc diim vi hemoglobin, ferritin cia bfnh nhin thalassemia thi nfng cd lin thuang lim mpch vi khdng tin thuong tim mpch
Nhom cd ton thuang tim m^ich thiiu miu ning hon thuong tim va ferritin b-ung binh cao hon hin nhdm khdng ton thuong
tim mteh vii ferritin > 2500 ng/ml chiim din 40,4% (21/S2).
Bfc diim vi tlin sir Otiu trj cua bfnh nhin Ihttlttssemitt thi nfng cd lin thuang tim mpch vi khing tin thucmg tim mpch
Nhdm tin thuong tim m^h cd si liin hniyin miu trong nim va ti If thii sit thip hon so vdi nhdm khdng t6n thuong tim m^h trii l(u ti If cit lich ttong nhdm tin thuong tim m^h l(ii cao hon nhdm khdng tin thuong.
V. Kb mk**
Ti if tin thuong tim m^ch li 83,9%
trong dd din buing tim, ting ip phii, phi d^
thit ttii, suy tim li b6n lo«i tin thuong thudng gip nhit. Cic lo^ tin thuong cd the ching lin len nhau, suy tim vi ting ip phii thudng xuit hifn ding thdi. Nhdm tin thuong tim m^ch cd tuii nhd hon, phit hifn bfnh vi truyin miu sdm hon, da si thuQC the P thalassemia; ti If khdng do cha m« nudi dudng cao, hoin cinh kinh ti khd khin hon;
bfnh nhin thieu miu ning vi li sit nhiiu hon nhung ti If truyin miu vi thii sit tliip hon.
VI. KII'N NGH|:
Nin tim soit sdm tin thuong tim m^h cho bfnh nhin thalassemia thi ning di cd ki ho^ch diiu trj sdm, tich eye.
- Cic bfnh vifn tuyin tinh cd thi theo ddi diiu b^ cho bfnh nhan tlialassemia thi ning bong giai d o ^ diu bing truyin hing ciu ling, thii sit t^i dja phucmg.
- Bfnh vifn tuyin trung uong cin du^ic trang bj phuong tifn di ting cudng tam soit cic ton thuang tim mjch tren bfnh nhin thalassemia thi ning cung nhu eic bien chiing idlic cd lien quan vdi tinh trang ii sit cua bfnh nhan
- Nen cd nhihig nghien ciiu tiip theo vdi 74
Y HQC VI6T NAM THANO 8 • s g DJiC BlgTQOtZ Cd miu Idn hon v i biin chiing tim msich ciia
bfnh nhin thalassemia thi ning.
TAI u l u THAM KHAO
1. Arshad M.S. and S.N. Hyder, (2009), "Evidence of abnormal lei^
ventricular function in patients with thalassaemia major: an echocardiography based study". JAyub Med Coll Abbottabad, 21(2), pp. 37-41.
2. Aessopos, A., M. Kati, and M. Tsironl, (2008), "Congestive heart failuie and treatment in thalassemia major".
Hemoglobin, 32(1-2), pp. 63-73.
3. Bili Vin ViSn, NguySn Cing Khanh (1999), Mpt s6 die diim idm sing huyet hpc benh hemoglobin va tin suit ngudi mang gen hemoglobin E a dm dan tdc Mudng Hoa Thanh, Luin in Tien sT Y hpc, trudng
dai hoc Y Hi Nfi, Hi Ndi.
Ehlers KH, Markenson AL, et al, (1980),"
Longitudinal study of cardiac iunction in thalassemia major". Ann NY Acad Set, 344(397-404).
Lim Thj M ; v i U Bich Liin, (2003),"Tlnh hlnh chin doin vi diiu bj b$nh thalassemia tai binh viin Nhi Ding I".
Tpp chi y hpc thinh phi Hi Chi Minh, php ban si 1, 7, b.39-42.
Liu P (1994)," Iron overload cardiomyopathies: new ninsights into an old disease. "Cardiovasc Drugs Ther, 8, pp.
101-110.
M i Phinmg H;nh, (2009), "Die diim bfnh nhin thalassemia the ning c6 li sit tai bfnh vien Nhi Ding I", Tpp chiy hpc thinh phi Ho Chi Minh, phu bin tip 13 so I, chuyen di Nhi Khoa, b.167-173.
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