Kan zingah hian natna chi hrang hrang a awm a, chubakah natna chi hrang hrang, chu chu a tlem hle tihna a ni. Chutiang natna chu i la hre lo pawh a ni mai thei. Vawiin hian hetiang natna hlauhawm tak, mahse thisen kaihhnawih pawimawh tak, uluk taka enkawl a pawimawh chungchang kan sawi dawn a ni. A hming chu Paroxysmal Nocturnal Hemoglobinuria a ni a, a tawi zawngin PNH kan ti a.
A awlsam zawngin PNH chu eng nge ni?
PNH hi genetic disorder a ni lo va, nu leh pa atanga rochun emaw, fate hnena kai emaw a ni. Kum eng pawhah a lo thang lian thei. A awlsam zawngin, he natna vei hian natna a nei a, chutah chuan
immune system , natna laka min humhimtu system chuan anmahni thisen sen (red blood cells) chu a rawn bei sual a, a tichhe vek a ni. Natna laka min vengtu protein bik hmanga siam phaw ang maiin han ngaihtuah teh. PNH vei taksaa thisen sen thenkhat chuan he venhimna hi an nei lo, chu chu chutiang protein bikte chu a ni. Chuvangin immune system chuan heng cell te hi shield nei lovin a hre chiang a, taksa tana invader tha lo takah a ngai a, a tichhia a ni. Hetiang dinhmun hi nunna atana hlauhawm tak ni thei mah se,
enkawl dan dik tak chuan a natna lan chhuah dan chu i thunun thei a, harsatna i neihte chu i ti tlem thei a, nun tha tak i hmang thei bawk. He natna hian mi zawng zawng a nghawng dan inang vek lo. Mi thenkhat chuan harsatna tenau te chauh an nei thin. Mi dangte chu nasa zawkin an tuar thei bawk.
A hlauhawm ber chu thisen zam (blood clots) a ni. PNH vei zinga 30% vel chu an dam chhungin vawi khat tal thisen zam (blood clot) an nei thei a ni.
Hei hi eng nge a chhan?
He natna thlentu hi gene pakhata mutation a ni. Mahse hei
hi rochun thil a ni lo. Chumi awmzia chu i nu leh pate hnen atanga i dawn thil a ni lo va, i fate hnena i hlan chhawn pawh a ni lo. Hei hi dam chhunga lo awm random genetic change a ni. He gene mutation avang hian i ruhro hian thisen sen danglam tak tak a siam tan a, chu chuan chutiang venhimna proteins chu a nei lo. Chuvangin heng cell te hi immune system in a humhim loh avangin awlsam takin an chhe vek a ni. Damdawi lamah chuan he thisen zam (red blood cell breakdown) kalphung hi
hemolysis tiin kan sawi thin. Doctor thenkhat chuan PNH hi ruhro chak lohna nen a inzawm niin an ngai. A bik takin aplastic anemia tih natna nei te hian PNH an vei tam zawk. Tin, PNH vei te hian a hnuah aplastic anemia an nei thei bawk. Hetiang dinhmun aplastic anemia an tih hian ruhro hian thisen cell thar a siam chhuak tawh lo.
PNH natna lan chhuah dan chu engte nge ni?
He natna hi a chhinchhiahna ber pakhat hming chawia phuah a ni. Paroxysmal tih awmzia chu "rang taka lo thleng" tihna a ni a, Nocturnal tih awmzia chu "zanlai" tihna a ni a, Hemoglobinuria tih awmzia chu "zun atanga hemoglobin paih chhuah" tihna a ni. Hemoglobin hi thisen rawng sen petu protein a ni. Chuvângin, thisen sen (red blood cells) a chhiat chuan he hemoglobin hi zun-ah a chhuak a,
zan emaw, zing emawa zun chhuak chu a dum (cola ang chi) emaw, a sen emaw a ni thei . PNH vei zinga 50% vel chuan he natna hi an nei thin. A natna lan chhuah dan hi chhan pathum vang a ni ber a:
I taksaa thisen cell chhia i neih tam poh leh symptom i nei tam zawk thei.
Thisen zam (red blood cells) a chhe leh thisen tlakchham (anemia) avanga natna lan chhuah dan tlangpui
| Chau leh chak lo tak nia inhria | Hna tlemte thawh hnu pawha chau tih hriat. |
| Lu na | Lu na fo thin. |
| Thâwk harsa | Hmun hla vak lo kal pawhin thawk hah. |
| Lung na tak tak | I thinlung chu a na hle ang tih hriatna. |
| Pum a na | Chhan awm lovin pum na. |
| Chaw ei harsat | Chaw emaw tui emaw ei harsa. |
| Vun dum emaw, sen emaw | Vun sen leh mit var. |
| Bruising awlsam tak a ni | Hliam tenau tak pawh hian hliam na tak a thlen thei. |
| Mipaah sexual dysfunction a awm thin | Mipat hmeichhiatna hman laiin arousal neih emaw vawn reng emaw harsatna. |
Pawimawh tak: Thisen zam hi natna hlauhawm tak a ni a, damdawi lam pan nghal ngai a ni. A hnuaia symptoms te hi fimkhur hle ang che.
| Thisen zam awmna hmun | Features ngaihven tur a awm |
|---|
| Vun | Vun a sen, na, emaw, a hring emaw. |
| Arm emaw Leg emaw | Kut/ke na, lumna, leh hring. |
| Pumpui | Pum natna nasa tak, ulcer, leh thisen chhuak. |
| Thluak | Lu na tak tak, luak chhuak emaw awm lo emaw, seizures, che harsa, tawng leh hmuh harsa. |
| Chuap | Thâwk harsa, chemte ang maia rilru na tak, thisen khuh, thawk hah. |
Hetiang thisen zam i nei tih rinhlelhna tlemte pawh i neih chuan,
I doctor hnenah hriattir nghal rawh. Damdawi in hnai ber Emergency Department (ETU)-ah tlai lovin kal la, a bik takin thawk harsa, tawng/ mit harsatna, lu na tak tak, rilru na, emaw, seizures i neih chuan. Engtin nge natna hi dik taka kan hriat theih ang?
Doctor hmu tura i kal hunah i symptoms leh medical history te a zawt ang. Tichuan a enfiah ang che a, thisen test engemaw zat a order ang. Chumi atana test tih ber chu thisen test bik flow cytometry tih hi a ni . Hei hian i thisen sen (red blood cells) te hian heng venhimna protein te hi an neih theih nan a pui a ni. Tin, i thisen cell count (full blood count) leh iron level te pawh enfiah a ni bawk. A chang chuan bone marrow sample lak a ngai bawk. PNH enkawl dan tur chu engte nge ni?
PNH enkawlna kawngah hian tum lian tak pahnih a awm a. Pakhat chu symptoms tihziaawm nan a ni a, pakhat zawk chu harsatna hlauhawm (a bik takin thisen zam) ven nan a ni. I enkawlna tur chu i dinhmun nasat danah a innghat ang.- Additional nutrients: Anemia control nan folic acid leh iron supplement pek thin a ni.
- Damdawi bik: Thisen sen (red blood cells) tihchhiat lohna tur damdawi Eculizumab (Soliris) leh Ravulizumab (Ultomiris) te a awm a. Chungte chu thisenah pek thin a ni. Anni hian thisen tlakchhamna (anemia) thunun theihna te, pawn lam thisen pek ngai lohna te, thisen zam awm theihna a tihziaawmna te an nei a ni. Hemi atana hman damdawi thar zawk dang chu Pegcetacoplan (Empaveli) a ni.
- Thisen pek: Thisen tlakchhamna a nasat chuan mi hrisel hnen atanga thisen pein hun eng emaw chen a hahdam theih a ni.
- Blood thinners: Thisen tithianghlimtu hi thisen zam hlauhawmna sang tak nei te hnenah pek thin a ni.
- Bone Marrow Stem Cell Transplant : Hei hi PNH tihdam kim theihna awmchhun a ni. Hei hian ruhro natna nei chu lakchhuah a, mi hrisel (a tam zawkah chuan unau) hnen atanga stem cells thlak a huam a ni. Mahse, hei hi thil hlauhawm leh buaithlak tak a nih avangin natna khirh tak nei thalai tan chauh a tha tlangpui.
I doctor chuan eng enkawlna kawng nge i tan a tha ber tih a rel ang. Mahni inenkawl a pawimawh hle bawk.
PNH nena kan awm hian a hma aia nasa zawkin mahni in enkawl hi a pawimawh zawk.- Ei leh in tha: Chaw tha tak tak ei thin ang che. Iron tamna ei tur (spinach, sa) ei rualin vitamin C tamna thil (orange, tangerines) ei hian taksain iron a hip lut tha zawk thin.
- Exercise: Chauhna hian exercise lak a ti harsa thei hle. I doctor hnenah eng exercise nge i tan a tha tih zawt rawh.
- Infection laka invenna : Kut sil fo thin ang che. Mipui tamna hmun leh damlo awmna hmunte chu pumpelh tur a ni. Khawsik i neih chuan doctor hnenah hrilh nghal rawh.
- Rilru hriselna: Hetiang natna rare tak i nei tih i hriat hian lungngaihna, thinrim leh lungkhamna i neih hi thil pangngai tak a ni. Hêng rilru put hmang chungchâng mi rintlak emaw, counselor emaw nêna sawiho chu thlamuanna nasa tak a ni thei a ni.
- Naupai: Hmeichhia naupai tum i nih chuan naupai hmain doctor rawn hmasa phawt ang che. PNH hian naupai laiin nang leh i naute tan hlauhawmna a thlen thei a ni.
Take-Home Thuchah a ni
- PNH hi thisen lama harsatna awm lo, genetic mutation avanga lo awm a ni a, mahse rochun a ni lo.
- He natna complication ber leh hlauhawm ber chu thisen zam hi a ni. A vaukhânna chhinchhiahna (lu na nasa tak, rilru na, thawk harsat) te chu hre reng ang che.
- Hei hi nunna atana hlauhawm tak ni thei mah se, tunlai hian natna lan chhuah dan control leh nunphung tihchangtlunna tur enkawlna tha tak tak a awm a.
- PNH hi bone marrow transplantation chauh hi fully curative treatment ni mahse, risk sang tak a ni a, damlo thlan bikte tan chauh a tha hle.
- Doctor nen inbiak reng a, a thupek zawm reng a pawimawh hle.
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