Muaj cov kab mob uas peb pom ntau heev, thiab cov kab mob uas tsis tshua muaj, uas yog, tsis tshua muaj heev. Tej zaum koj tsis tau hnov txog cov kab mob zoo li no. Hnub no peb yuav tham txog cov kab mob ntshav uas tsis tshua muaj, tab sis tseem ceeb uas yuav tsum tswj hwm kom zoo. Nws lub npe yog Paroxysmal Nocturnal Hemoglobinuria, uas peb hu ua PNH luv.
Hais yooj yim xwb, PNH yog dab tsi?
PNH tsis yog ib qho mob caj ces, tab sis yog ib yam kab mob uas tau txais los ntawm niam txiv lossis kis mus rau cov menyuam. Nws tuaj yeem tshwm sim thaum twg los tau. Hais yooj yim, ib tus neeg uas muaj tus kab mob no muaj tus kab mob uas
lub cev tiv thaiv kab mob , lub cev uas tiv thaiv peb ntawm tus kab mob, ua yuam kev tawm tsam thiab rhuav tshem lawv cov qe ntshav liab. Xav txog nws zoo li lub tsho tiv thaiv ua los ntawm cov protein tshwj xeeb uas tiv thaiv peb ntawm tus kab mob. Qee cov qe ntshav liab hauv lub cev ntawm cov neeg mob PNH tsis muaj lub tsho tiv thaiv no, uas yog, cov protein tshwj xeeb. Yog li lub cev tiv thaiv kab mob paub cov qe ntshav no yam tsis muaj lub tsho tiv thaiv, xav tias lawv yog tus neeg ua phem rau lub cev thiab rhuav tshem lawv. Txawm hais tias tus mob no tuaj yeem ua rau tuag taus,
nrog kev kho mob zoo, koj tuaj yeem tswj cov tsos mob, txo cov teeb meem thiab ua neej zoo. Tus kab mob no tsis cuam tshuam rau txhua tus tib yam. Ib txhia neeg tsuas muaj kev tsis xis nyob me ntsis xwb. Lwm tus tuaj yeem raug cuam tshuam los ntawm nws hnyav dua.
Qhov kev pheej hmoo tseem ceeb yog cov ntshav txhaws. Kwv yees li 30% ntawm cov neeg mob PNH muaj kev pheej hmoo ntawm kev tsim cov ntshav txhaws tsawg kawg ib zaug hauv lawv lub neej.
Vim li cas ho ua li no?
Qhov ua rau muaj tus kab mob no yog kev hloov pauv hauv ib hom noob caj noob ces. Tab sis qhov no
tsis yog ib yam dab tsi uas tau txais los ntawm noob caj noob ces. Qhov ntawd txhais tau tias nws tsis yog ib yam dab tsi uas koj tau txais los ntawm koj niam koj txiv, thiab tsis yog ib yam dab tsi uas koj xa mus rau koj cov menyuam. Qhov no yog kev hloov pauv noob caj noob ces uas tshwm sim thaum lub neej. Vim yog qhov kev hloov pauv noob caj noob ces no, koj cov pob txha pib tsim cov qe ntshav liab tsis zoo uas tsis muaj cov protein tiv thaiv. Yog li ntawd, txij li thaum cov qe ntshav no tsis raug tiv thaiv los ntawm lub cev tiv thaiv kab mob, lawv yooj yim tawg. Hauv kev kho mob, peb hu qhov txheej txheem no ntawm kev rhuav tshem cov qe ntshav liab
hemolysis . Qee tus kws kho mob ntseeg tias PNH muaj feem cuam tshuam nrog qhov tsis muaj zog hauv cov pob txha. Tshwj xeeb, cov neeg uas muaj mob hu ua aplastic anemia yuav muaj feem ntau yuav mob PNH. Tsis tas li ntawd, cov neeg uas muaj PNH tuaj yeem mob aplastic anemia tom qab. Hauv qhov mob no hu ua aplastic anemia, cov pob txha tsis tsim cov qe ntshav tshiab.
Cov tsos mob ntawm PNH yog dab tsi?
Tus kab mob no muaj npe raws li ib qho ntawm nws cov tsos mob tseem ceeb. Paroxysmal txhais tau tias "tshwm sim tam sim ntawd", Nocturnal txhais tau tias "thaum hmo ntuj", thiab Hemoglobinuria txhais tau tias "kev tso hemoglobin tawm hauv cov zis". Hemoglobin yog cov protein uas ua rau cov ntshav muaj xim liab. Yog li, thaum cov qe ntshav liab tawg, cov hemoglobin no raug tso tawm rau hauv cov zis,
thiab cov zis tawm thaum hmo ntuj lossis thaum sawv ntxov tuaj yeem tsaus (zoo li cola) lossis liab . Kwv yees li 50% ntawm cov neeg uas muaj PNH muaj cov tsos mob no. Cov tsos mob feem ntau yog tshwm sim los ntawm peb qho laj thawj:
Koj cov qe ntshav tsis zoo ntau npaum li cas hauv koj lub cev, koj yuav muaj cov tsos mob ntau npaum li ntawd.
Cov tsos mob tshwm sim los ntawm kev tawg ntawm cov qe ntshav liab thiab ntshav tsis txaus
| Xav tias nkees heev thiab tsis muaj zog | Txawm tias ua ib txoj haujlwm me me los xij, koj yuav xav tias nkees. |
| Mob taub hau | Mob taub hau ntau zaus. |
| Ua pa nyuaj | Ua pa nrov nrov txawm tias taug kev luv luv xwb los xij. |
| Lub plawv dhia ceev | Zoo li koj lub plawv dhia ceev. |
| Mob plab | Mob plab tsis muaj laj thawj. |
| Nyuaj nqos | Nyuaj nqos zaub mov los yog kua dej. |
| Tawv nqaij daj los yog daj | Tawv nqaij thiab qhov muag dawb daj. |
| Mob nqaij yooj yim | Txawm tias raug mob me me los kuj tuaj yeem ua rau muaj kev mob hnyav. |
| Kev tsis muaj peev xwm sib deev ntawm cov txiv neej | Nyuaj siab ua kom tau lossis tswj tau qhov kev xav thaum lub sijhawm sib deev. |
Tseem ceeb heev: Cov ntshav txhaws yog ib qho mob txaus ntshai uas yuav tsum tau kho mob thaum muaj xwm txheej ceev. Yuav tsum paub txog cov tsos mob hauv qab no.
| Qhov chaw ntawm cov ntshav txhaws | Cov yam ntxwv uas yuav tsum tau saib xyuas |
|---|
| Tawv nqaij | Ib qho chaw liab, mob, lossis o ntawm daim tawv nqaij. |
| Caj Npab los yog Ceg | Mob caj npab/ceg, xav tias sov so, thiab o. |
| Plab | Mob plab hnyav, mob qog, thiab los ntshav. |
| Lub hlwb | Mob taub hau hnyav nrog lossis tsis ntuav, qaug dab peg, nyuaj txav, hais lus, lossis pom kev. |
| Lub ntsws | Ua pa nyuaj, mob hauv siab ntse zoo li riam, hnoos tawm ntshav, tawm hws. |
Yog koj muaj qhov kev xav me ntsis tias koj muaj cov ntshav txhaws zoo li no,
Qhia rau koj tus kws kho mob tam sim ntawd. Mus rau chav kho mob ceev (ETU) ze tshaj plaws yam tsis muaj kev ncua, tshwj xeeb tshaj yog tias koj ua pa nyuaj, hais lus/pom tsis meej, mob taub hau hnyav tam sim ntawd, mob hauv siab, lossis qaug dab peg. Yuav ua li cas thiaj kuaj tau tus kab mob kom meej?
Thaum koj mus ntsib koj tus kws kho mob, nws yuav nug txog koj cov tsos mob thiab keeb kwm kev kho mob. Tom qab ntawd nws yuav kuaj koj thiab xaj qee qhov kev kuaj ntshav. Qhov kev kuaj tseem ceeb uas ua rau qhov no yog kev kuaj ntshav tshwj xeeb hu ua flow cytometry . Qhov no yog qhov ua kom paub tseeb tias koj cov qe ntshav liab muaj cov protein tiv thaiv no. Tsis tas li ntawd, koj cov qe ntshav suav (tag nrho cov ntshav suav) thiab cov hlau kuj raug kuaj xyuas. Qee zaum yuav xav tau cov qauv pob txha. Cov kev kho mob rau PNH yog dab tsi?
Muaj ob lub hom phiaj tseem ceeb ntawm kev kho mob PNH. Ib qho yog kom txo cov tsos mob, thiab qhov thib ob yog kom tiv thaiv cov teeb meem txaus ntshai (tshwj xeeb tshaj yog cov ntshav txhaws). Kev kho mob uas koj tau txais yuav nyob ntawm seb koj tus mob hnyav npaum li cas.- Cov khoom noj ntxiv: Muab cov tshuaj folic acid thiab hlau los tswj cov ntshav tsis txaus.
- Cov tshuaj tshwj xeeb: Muaj cov tshuaj xws li Eculizumab (Soliris) thiab Ravulizumab (Ultomiris) uas tiv thaiv kev tawg ntawm cov qe ntshav liab. Cov no yog muab rau hauv cov hlab ntsha. Lawv muaj qhov zoo ntawm kev tswj cov ntshav tsis txaus, txo qhov xav tau kev hloov ntshav sab nraud, thiab txo qhov kev pheej hmoo ntawm cov ntshav txhaws. Pegcetacoplan (Empaveli) yog lwm yam tshuaj tshiab siv rau lub hom phiaj no.
- Kev Hloov Ntshav: Yog tias tus neeg mob ntshav tsis txaus, kev pab ib ntus tuaj yeem tau los ntawm kev pub ntshav los ntawm ib tus neeg noj qab haus huv.
- Cov tshuaj ua kom cov ntshav nyias: Cov tshuaj ua kom cov ntshav nyias yog muab rau cov neeg uas muaj feem yuav ntshav txhaws ntau.
- Kev Hloov Cov Qog Pob Txha: Qhov no yog tib txoj kev los kho PNH kom zoo . Qhov no suav nrog kev tshem tawm cov qog pob txha uas muaj mob thiab kev hloov cov qog ntawm tus neeg noj qab haus huv (feem ntau yog tus kwv tij). Txawm li cas los xij, vim tias qhov no yog ib qho kev phais mob uas muaj kev pheej hmoo thiab nyuaj heev, feem ntau nws tsuas yog pom zoo rau cov tub ntxhais hluas uas muaj mob hnyav heev.
Koj tus kws kho mob yuav txiav txim siab seb txoj kev kho mob twg zoo tshaj plaws rau koj. Nws tseem ceeb heev rau koj saib xyuas koj tus kheej.
Thaum nyob nrog PNH, nws tseem ceeb dua puas tau los saib xyuas koj tus kheej.- Kev Noj Haus Zoo: Noj cov khoom noj muaj txiaj ntsig zoo. Kev noj cov khoom noj uas muaj hlau ntau (zaub paj ntsuab, nqaij) nrog rau ib yam dab tsi uas muaj vitamin C ntau (txiv kab ntxwv, txiv kab ntxwv qaub) pab lub cev nqus tau hlau zoo dua.
- Kev Ua Si: Kev qaug zog ua rau nyuaj rau kev ua si. Nug koj tus kws kho mob seb kev ua si twg yog qhov zoo rau koj.
- Tiv thaiv koj tus kheej kom tsis txhob muaj kab mob : Ntxuav koj txhais tes ntau zaus. Tsis txhob mus rau qhov chaw muaj neeg coob coob thiab cov chaw uas muaj neeg mob nyob. Yog tias koj kub taub hau, qhia rau koj tus kws kho mob tam sim ntawd.
- Kev noj qab haus huv ntawm lub hlwb: Nws yog ib qho ib txwm muaj uas koj yuav tu siab, npau taws, thiab ntxhov siab thaum koj paub tias koj muaj ib yam kab mob tsis tshua muaj li no. Kev tham txog cov kev xav no nrog ib tus neeg uas koj ntseeg siab lossis tus kws pab tswv yim tuaj yeem pab tau koj zoo heev.
- Cev Xeeb Tub: Yog tias koj yog ib tug poj niam uas tab tom npaj yuav cev xeeb tub, nco ntsoov sab laj nrog koj tus kws kho mob ua ntej cev xeeb tub. PNH tuaj yeem ua rau muaj kev pheej hmoo rau koj thiab koj tus menyuam thaum cev xeeb tub.
Cov Lus Qhia Txog Kev Coj Mus Tsev
- PNH yog ib qho kab mob ntshav tsis tshua muaj uas tshwm sim los ntawm kev hloov pauv caj ces, tab sis tsis yog los ntawm noob caj ces.
- Qhov teeb meem loj tshaj plaws thiab txaus ntshai tshaj plaws ntawm tus kab mob no yog cov ntshav txhaws. Nco ntsoov paub txog nws cov cim ceeb toom (mob taub hau hnyav, mob hauv siab, ua pa nyuaj).
- Txawm hais tias qhov no tuaj yeem ua rau muaj kev phom sij rau lub neej, tab sis niaj hnub no muaj kev kho mob zoo heev los tswj cov tsos mob thiab txhim kho lub neej zoo.
- Txawm hais tias kev hloov pob txha yog tib txoj kev kho mob zoo rau PNH, nws muaj kev pheej hmoo siab thiab tsuas yog tsim rau cov neeg mob xaiv.
- Nws yog ib qho tseem ceeb heev uas yuav tsum sib cuag nrog koj tus kws kho mob tsis tu ncua thiab ua raws li nws cov lus qhia.
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Tsis muaj lus teb tau muab tso tawm. Ntxiv koj cov lus tawm ntawm no thawj zaug.
Ntxiv koj cov lus