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ONKOLOGIE3418040010760120167227269329388409453
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Onkologie.9168040010761110147195214263313331375390
2024;18(5):330-3331326804001074487128168182220259283325348362407448492522563604645
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www.onkologiecs.cz48658040010765133201214262311348396415464514532576617652669709748
3301278020012657113170
SDĚLENÍ341200012656130188244301376409
Z772200012659
PRAXE853200012660123195263321
Emicizumab34136001063084102136154187228281322357
v714360010636
léčbě7663600106296196132164
získané9463600106314881116158199230
hemofilie119236001063971126166197215246263295
A1503360010638
–1556360010645
naše161736001063979113144
první177736001063368106147165
zkušenost195836001063066107139171212253286318
Emicizumab3409200298128334398504569681820102411501291
v16809200298127
léčbě1856920029866191299442567
získané24729200298113179281410536674799
hemofilie332092002981402654716086897578218841009
A43789200298157
–45849200298120
naše34011800298141265367493
první88111800298144234365504568
zkušenost1498118002981122433824856107508879891075
Eva3401580013759116174
Drbohlavová5361580013776119184248313343401457519575633
Oddělení3401740013376139200255281336397422
klinické785174001335279103164189237288341
hematologie,11501740013361116207260295354380440502527582603
Krajská1777174001335996149175217268321
nemocnice21221740013361116207268317378402451505
Liberec2650174001335277139195230285334
Získaná340225001215577115160208263310
hemofilie6682250012156105188242270295317339389
A10752250012161
(AHA)1155225001212888151211238
je1411225001212574
vzácné1503225001214992141185239289
krvácivé1810225001214780131178222244291339
onemocnění21682250012155109159241296340395444498520
vyskytující2707225001214898136182231266320343365409431
se3156225001214089
především3264225001215789137194243290328350432
ve3715225001214896
vyšším340239001214898136174196278
věku.638239001214896142197217
Je874239001213787
způsobená981239001214399153191245301351406453
přítomností1454239001215789111143196279333387425458480
autoprotilátek19542390012148102134187243275329361383405452483532578
proti2552239001215688142174196
koagulačnímu2768239001214799147202256278325369423445527581
faktoru3369239001212977123158211243297
VIII3686239001215679102125
(FVIII).340253001212875132155178201227245
Může6052530012181134176225
být8502530012157102138
asociována1008253001214886140185207261307355409456
s14842530012140
autoimunitním15442530012148102134187209291345399421454508530612
onemocněním,21762530012155110159242296341395444499521603623
malignitou28192530012184131153175230285307339392446
či3285253001214567
po33722530012157112
porodu35042530012157112144197254307
nebo3402670012155104160214
vzniká570267001214791145167212260
idiopaticky.845267001212378100154209257288309353398446462
Důležitá13222670012166120142191232254286334
je1671267001212473
včasná1759267001214890137175229275
diagnóza2050267001215678124179232286326375
a24402670012148
léčba,2504267001212372116171218238
která2758267001214681128160207
spočívá2981267001213994148192214261308
v33042670012148
léčbě3368267001212372116171221
krvácení3604267001214779129176340281001214593147170
a5272810012148
imunosupresivní5932810012123105159213268306359415447496534556603659681
léčbě1291281001212473118173223
k15322810012147
navození15962810012156103148201242291345368
remise1981281001213382164186224274
onemocnění22722810012155110159242296341395444499521
čili281028100121456789111
k29392810012147
eradikaci3003281001215082130185207253301345367
inhibitoru.3387281001212478132154210233265318350404424
Zmiňujeme3402950012155138160215269292342425475
případ840295001215789112168216273
pacientky11372950012157105150172222277309355405
s15662950012140
revmatoidní1630295001213382129213260292346369425480502
artritidou2157295001214880115147170203225281336391
s25722950012140
krvácivými2636295001214781132180224246294344428451
projevy3111295001215790144167217264314
z34502950012142
gastrointestinálního35172950012156103142175207261340309001212378110159198231254308356379433456513567
traktu927309001213365114160195249
(GIT),1197309001212890114164194212
kožními14293090012147101142197220302325
sufuzemi1774309001214094123178221270353376
a21703090012148
protrahovaným2239309001215689143175207255310365411459514559643
krvácením2902309001214780131179223272327350432
po33553090012157112
extrakci3487309001215097132164213259302324
zubu.340323001214397154209229
U5863230012165
pacientky6693230012156105149172221276308354404
byla10903230012157103127174
diagnostikována1282323001215679126182237291330363386432485532580635682
AHA19813230012162126186
a21843230012149
bylo22503230012157103127181
nutné24493230012155109142197247
zahájit2713323001214392147195218241273
léčbu3003323001212474119175230
rekombinantním3250323001213382129182265322345400447502534340337001215578161
aktivovaným518337001214894129152200253300347402447531
faktorem1066337001212977123159212245294377
VII1460337001215680103
(rFVIIa).1580337001212958106163187210258283301
Vstupně1898337001215692126180236291341
zjištěn225633700121436689127160209264
vysoký2537337001214898137192238288
inhibitor,2842337001212378133155211235267321353368
který3227337001214783131164213
po34573370012157113
léčbě3587337001212373118174224
kombinované340351001214698179234256310363408455508557
imunosuprese9133510012123103156210263301353408439487525574
(kortikoidy,1503351001212872124156190212256308330385431446
cyklofosfamid,1964351001214492138161215242295333362409490511566585
rituximab)256535100121325486139184207287334389413
klesal2993351001214770119157204226
velice3234351001214895118139182230
pomalu.34793510012156110191238260313332
Pro340365001215384139
nově5063650012155110157205
zjištěné73836500121436789127161209264314
krvácení1079365001214781132180224273328350
do14573650012156111
musculus15953650012183138176221275298352391
psoas2013365001215796150199237
byla22773650012157103127174
navýšena24793650012155103149199238288343390
substituce2897365001213993149188222244277331376424
rFVIIa3348365001213381138163186234
a36093650012148
byl36853650012157102126
indikován340379001212378134157203256303351406
emicizumab,7673790012150133156200222265319402450506525
k13133790012147
prevenci1381379001215789139186234290334357
dalšího17593790012156104126165187244299
krvácení2079379001214780131179223272327349
u24503790012155
pacientky25263790012157105149172221277308354404
s29513790012140
přetrvávajícím3012379001215789139171204254302348396419442486509591
inhibitorem.3625379001212378133155340393001215780113166199248331352
Díky710393001216790138188
jeho917393001212474129184
s.1120393001213960
c.1192393001214465
aplikaci12763930012148104128151197245290312
mohla16073930012183138193216264
být18893930012157103139
pacientka20473930012157105149172221277308354403
propuštěna2469393001215789143200255293327375430478
do29653930012157111
ambulantní30953930012148131187242265313367399454476
péče.35903930012157108153201221
Klíčová340409001246189117161218270322
slova:679409001244371128180232257
získaná954410001214366104150198253301
hemofilie12724100012155105188243271297320343392
A,1682410001216183
bypassové17834100012156102159208246285340386435
přípravky,2235410001215789112169201249295342392408
imunosuprese,26604100012123106160215269308362418451500539589609
emicizumab.32864100012150133156200223265319402450506525
Emicizumab3404370012453140168213242289348434488548
in908437001242886
treating1015437001243675129182219247306365
acquired1400437001245398158216244283336396
haemophilia18174370012458111164251309370429456484512565
A:2402437001246692
our25144370012458116155
initial2689437001242786114151179232260
experience29694370012453106167220259286339399444497
Acquired3584550012161104159213235267317373
haemophilia7484550012156103153235290346401424447469517
A12824550012161
(AHA)1360455001212888152212239
is1616455001212462
a16954550012148
rare1760455001213381113162
bleeding1939455001215780130181237260314370
disorder2326455001215679117172204260309342
occurring26854550012155100143197229261284339394
particularly30974550012156104136172194239293316363395418465
in3579455001212478
the3675455001213287137
elderly.340469001215074131181214238285301
It662469001212457
is739469001212462
caused822469001214593148187237295
by11374690012157104
the1261469001213388139
presence1420469001215790140179230286331380
of1820469001215584
autoantibodies19254690012148102135189238293326349405461519542592631
against25774690012148104152175231269303
coagulation2901469001214498147203258281329362385440495
factor3417469001212978123158212245
VIII3683469001215580104128
(FVIII).340483001212875131155178201228245
It602483001212456
may6754830012182129174
be8664830012156106
associated989483001214785123178222244291323371427
with1433483001217295127181
an16314830012148102
autoimmune17504830012147101133186208290372425479529
disease,2296483001215578115165212250299318
malignancy,26314830012183130152174229283330384428477492
occur3140483001215499141195227
after3383483001214876112160192
birth,3592483001215679110145199219
or340497001215588
arise455497001214880104143193
idiopathically.675497001212380103158215264296352375419468491515562578
Early1280497001215098131155202
diagnosis1509497001215679127183239294333356395
and19314970012148103160
treatment2117497001213466116164196280330386418
are2562497001214881131
important,27194970012124107164220253289337393425446
which31924970012174129152197252
involves3471497001212379125179203251300340
treatment340511001213366115163196279330385418
of779511001215584
bleeding884511001215781131182240263318374
and12795110012148103160
immunosuppressive14605110012124107190245300355394449506563596646685724747795844
therapy2326511001213388138171220276323
to2670511001213387
induce2778511001212379135190235284
disease3083511001215680118169217256306
remission3410511001213383166190229268290345401
or340525001215588
eradicate449525001215083132188211256305337386
the857525001213388138
inhibitor.1016525001212479134157214238271325358374
We3405390012185131
report492539001213381137192224259
a7725390012148
case840539001214593131180
of1041539001215583
a11445390012149
female1213539001212978161208231280
patient15135390012157105137159208262294
with1827539001217497129183
rheumatoid2030539001213388137191274321352405428483
arthritis2534539001214879114168200222254276314
with2869539001217396128182
bleeding3072539001215780129179235257312367
manifestations34595390012184131185207235285323340553001213381113135189243281
from647553001213062116198
the872553001213387136
gastrointestinal10355530012156103141174205259281336367415454487509563610632
tract1694553001213364112156190
(GIT),1910553001212990114163193211
skin2147553001214085109163
suffusions,2336553001214094122154209247268323377415435
and27975530012149103159
prolonged2982553001215789142165220274330379435
bleeding3444553001215680129179235258312367
after340567001214876112160192
tooth553567001213386141172227
extraction.801567001215096131163210254288310364419439
The12615670012150105155
patient14375670012157105136158207262293
was17525670012173121159
diagnosed1932567001215678125180235289327376433
with2386567001217496128183
AHA25905670012161125184
and27965670012148102158
had29755670012155102158
to3154567001213386
be32615670012157107
started3390567001213972120151186235291
on37025670012155110
treatment340581001213363111157187268316369400
with754581001217395126179
recombinant948581001213279123174255310331384430483513
activated1476581001214890124145191237268315370
factor1860581001212976119152204235
VII2109581001215578100
(rFVIIa).2224581001212856102157180202248272289
Initially,252858100121237798129150196217239284298
a28415810012147
high2903581001215576130183
inhibitor3100581001212376129151205227258309340
level3455581001212372117164186
was36565810012172118155
found340595001212983137192247
that605595001213387134166
decreased7885950012157106151182231278316365422
very1227595001214896128178
slowly1422595001214062116188211257
after1697595001214876112160192
treatment1906595001213365114161192274324379410
with2334595001217396128182
combined2534595001214497180236258312362418
immunosuppression29705950012123105188241296350388441497553585634673711733787841
(corticoids,340609001212869122154189211255308331387425445
cyclophosphamide,811609001214594137159214270325379418474529576658681736786805
rituximab).164260900121335688142188211294341397422440
Due21086090012167122171
to2306609001213386
newly24186090012156106178201248
detected26926090012157106139187232267315372
bleeding3090609001215780130180237259313369
into3485609001212378109163
the3674609001213388137
psoas340623001215694148195232
muscle,5876230012183136174217239287306
rFVIIa908623001213380135159181228
replacement1151623001213280136158205248295377426479510
therapy1677623001213286135166213268312
was20056230012173119157
increased2177623001212377120151199245283332387
and25796230012148102157
emicizumab27516230012150131153196218259312393440495
was32616230012173120157
indicated3433623001212477132154197244275322378
to340637001213386
prevent446637001215789137184232287318
further784637001212984115150204254286
bleeding1089637001215780130180236258312368
in1476637001212478
the1574637001213387136
patient17306370012157105136158207262293
with2043637001217496128183
persistent22456370012157107139179200238271319374405
inhibitor.2670637001212378132154210232264317349365
Thanks30546370012150105152207252294
to3367637001213487
the3473637001213387137
subcutaneous3630637001213993148340651001214599131179233282337391429
route787651001213286140172221
of1025651001215583
administration,11256510012148104186208263285322355387434466488542597616
the1759651001213387136
patient19136510012156104136158207262293
could2223651001214598152174230
be24706510012157107
discharged2595651001215678116160214261293347397453
to3065651001213386
outpatient31696510012154109141197244276298347402433
care.3619651001214593124173192
Key3406670012461109157
words:5106670012475128164221262285
acquired808668001214889142193214243290344
haemophilia11656680012155100147227279333386406427447492
A,1670668001216081
bypassing1764668001215699154199236272292344397
agents,21746680012148100148200230267286
immunosuppression,24736680012123103182234286338375426479534564610647683703755808826
emicizumab.33126680012149129150192212252304383428482499
Úvod3407030014780141208279
do6467030014770138
problematiky8127030014770113182252284347449511553584645709
Získaná480720001095174112157205258305
hemofilie8217200010952101180233262289312336384
A12427200010957
(AHA1335720001092684146205
–15767200010946
acquired1659720001094588141194218250298353
haemophilia340734001095094140216267319370391413435479
A)843734001095580
je948734001092268
vzácné1040734001094384130171222268
krvácivé1332734001094274121165206228272317
onemocnění16737340010950101147223273315340748001095198151173
s5407480010937
incidencí604748001092274116138192239291334356
1,4987748001094863112
případů1126748001095284107160206259310
na1464748001095196
jeden1588748001092269123170223
milion18387480010977991221443407620010949100
obyvatel4647620010950102145192236266312334
za822762001093884
rok930762001092979123
dle1077762001095072118
dat1219762001095195125
z13687620010939
roku1430762001093080123174
2007,1628762001094692140185196
nyní1848762001094991143164
incidence340776001092274117140193241294337384
stoupá752776001093669120173227273
nejspíše10537760010952100123160214237277325
z14067760010939
důvodu14737760010952104150201256308
stoupajícího180977600109366912017321407060010953100124147190213268321
povědomí24957060010953107152199254307387410
o29397060010951
tomto3024706001093082162194246
onemocnění33037060010951105153233286330383432485508
na2140720001095196
cca2263720001094182127
624177200010947
případů2491720001095283106159204257308
na2826720001095196
jeden2949720001092269122169221
milion3198720001097698120142194246
obyvatel34717200010950103147195240271317340
za2140734001093985
rok.2252734001092980124145
Onemocnění24247340010963115161238289332383430481503
postihuje29547340010951103140171193244295317364
muže33457340010975126166212
i35847340010921
ženy3632734001093985137179
a21407480010944
může22127480010976128169216
se2455748001093784
vyskytnout2567748001094492129173221255307359411443
v30377480010945
kterémkoliv3109748001094377124155202280325375399421467
věku.3603748001094491135187208
Charakteristické21407620010952101145174218261293338368390425456477517559604
je2760762001092268
ale2844762001094365110
postižení29717620010951101137168189228273323344
vyššího3332762001094389125160181233283
věku3631762001094388130180
nad2140776001095093145
702301776001094691
let240877600109216798
či2522776001094162
v26007760010943
souvislosti2660776001093686136180202237259309345376397
s30747760010935
graviditou3126776001095080124166188239261291340390
typicky3533776001093075127149190232278
DECLARATIONS:4068227008648871301662142563043393604124625014570227008514
Declaration406824100874987120141179206244271291333375
of445724100874066
originality:45372410087406888130150192230251271298337356
The406825200853473109
manuscript418825200855891130168195227249266306329
is452825200851744
original45832520085386178117133172205222
and481625200853472112
has49392520085387299
not50502520085387699
been516125200853975110149
published532225200853977117134150177216251291
or406826300853862
submitted414526300852867108167184208234269310
elsewhere.44702630085355381117169209245269305320
Ethical406827700873764107127160198218
principles43002770087427090132165185228249287319
compliance:463327700873173134177197218256298330368387
The406828800853678116
authors420528800853475100142183208238
attest44642880085356087124153179
that466428800852465101126
their481128800852465103121146
study49782880085295595138173
was517328800855389118
approved531228800853577120145186221258301
by406829900854073
the41582990085236399
local42742990085175689124141
Ethical44322990085345898115147182199
Committee464829900854179139198215239265300337
and500229900853373113
is513229900851745
in519429900851656
compliance526729900853169129169187204239278311346
with40683100085537298140
human423631000854081143180221
studies44863100085285596139157195226
and474031000853577119
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