I zun a thim em? I chau reng em? PNH (Paroxysmal Nocturnal Hemoglobinuria) a ni thei bawk!

Zing i thawh hian i zun rawng pawh a danglam a, a sen dum emaw, a sen emaw, a chang chuan a dum emaw i nei em? A nih loh leh, nilengin chhan awm lovin i chau hle a, i chau hle em? Hengte hi kan nitin nunah kan ngaihven vak loh thil pangngai ang maiin a lang thei. Mahse, a châng chuan heng chhinchhiahna tenau te hi ngaihven tlem a ngai a, hriselna dinhmun hmuh tur awm lo mahse pawi tak a thup thei a ni. Vawiin hian chutiang thisen nena inzawm natna pakhat, PNH, emaw `(Paroxysmal Nocturnal Hemoglobinuria)` chungchang kan sawi dawn a ni. A hming hi a buaithlak deuhin a lang thei a, mahse awlsam takin i hrethiam ang u.

PNH chu eng nge ni? A awlsam zawngin i hrethiam ang u

Chuti a nih chuan, ``Paroxysmal Nocturnal Hemoglobinuria'' emaw PNH emaw chu eng nge ni? A nihna takah chuan thisen lama harsatna awm lo tak a ni. A hming awmzia i en chuan:

• "Paroxysmal" tih awmzia chu thil thleng nghal, a thleng nghal vek tihna a ni.
• "Zan lam" tih hian zan nena inzawm tihna a ni.
• "Hemoglobinuria" chu thisen protein pakhat ``Hemoglobin`` an tih chu zun chhunga awm a ni. He ``Hemoglobin`` hian kan thisen a sen tir thin. Chuvangin, hei hi zun nen a chhuah chuan zun chu a rawng a dum ta a ni.

A awlsam zawngin, PNH hi i immune system-in nangmah ngeiin i thisen sen (red blood cells) a bei a, a tihchhiat theihna dinhmun a ni . Doctor-te chuan hei hi ``Hemolysis`` emaw thisen sen (red blood cells) tihchhiatna emaw an ti a. Hei hi a lan chhuah dan ber pakhat chu zan emaw zingkar emaw i zun hian zun dum, sen/brown a ni.

Mahse, he zun dum hi PNH symptom pakhat chauh a ni. He natna hi enkawl tha a nih loh chuan natna khirh tak tak , thisen tlakchhamna , kal natna rei tak , emaw thisen kalna kawnga thisen zam te a thlen thei a ni. Vanneihthlak takin tunah hian PNH hian thisen cell a tihchhiat loh nan damdawi tha tak tak a awm ta.

Hei hi tuin nge tipung thei ang?

PNH hi natna awm lo tak a ni. Statistics atanga a lan dan chuan kum tin mi maktaduai khat zinga 6 velin he natna hi an vei thar an ni. A tam zawkah chuan he natna hi mipa leh hmeichhia kum 30 leh 40 inkar ah hmuh tur a ni a, mahse, hmeichhiate hian mipa aiin PNH an vei tam zawk niin an sawi bawk.

Thil dang pakhat chu, a chang chuan ruhro natna dang, entirnan, `(Aplastic Anemia)` emaw `(Myelodysplastic Syndrome)` ang chi natna nei te pawhin a hnuah PNH an nei thei bawk.

Hemoglobinuria leh Hematuria hi eng nge an danglamna ?

Heng thu pahnih ( confusion) i hriat hian i buai deuh a ni .- buaina, hei hi Tamil a ni a, ka pumpelh tur a ni. Ka sawi nawn leh ang: Heng thu pahnih i hriat hian i buai deuh mai thei.) Heng thu pahnih hi i hriat hian i buai deuh mai thei. A chhan chu a pahnih hian thil thleng chu zun chhungah thisen ang maia lang a awm a ni.

• Hematuria chu i zun chhunga thisen sen (red blood cells) awm vang a ni.
• Hemoglobinuria (hei hi kan PNH-a kan hmuh chu a ni) chu zun chhunga protein pakhat hemoglobin an tih awm hi a ni. Thisen sen (red blood cells) a chhiat chuan he hemoglobin hi a chhuak a, zun-ah a tel ve ta a ni.

A awlsam zawngin pakhat chu cell pum puiah a kal a, pakhat chu cell a chhiat huna thil sen lo chhuakah a kal bawk.

Eng thilin nge PNH hi a thlen? Engvangin nge hetiang hi a lo awm?

PNH awm chhan ber chu kan taksaa gene pakhat a chhiat vang a ni. He gene defect hian thisen cell chi hnih, red blood cell leh platelet te hnathawh a tichhe a ni. Hei hian thil thleng hrang hrang a tan a, chu chuan harsatna pakhat hnu pakhat a thlen a, a châng chuan nunna atana hlauhawm dinhmun pawh a thlen thei a ni.

Kan ruhro atang hian a intan vek a ni . I hria em, ruhro hi kan taksaa thisen factory ang mai a ni. Hei hi stem cell siamna hmun a ni. Heng stem cells te hi a hnuah thisen sen, thisen var, leh platelet ah te an lo thang lian a.

PNH-ah chuan ``PIGA`` an tih gene-a mutation emaw, inthlak danglamna emaw chu stem cell pakhatah a thleng a. He mutated stem cell hian a inthen darh a, chutiang bawkin stem cell chhia tam zawk a siam chhuak thin. Chumi hnuah chuan heng stem cell chhia te hian thisen sen leh platelet chhia te an siam chhuak leh thin.

He genetic change hian engtin nge thisen sen (red blood cells) a nghawng?

Hetiang hian han ngaihtuah teh: kan immune system hi kan ram defence force ang a ni. A chakna bik pakhat chu `Complement System` tih a ni. An hna chu taksaa lut natna hrik leh natna hrik dona a ni. Mahse, a chang chuan he `(Complement System)`-a protein te hian kan thisen sen hrisel tak takte chu tihsualin an rawn bei tan thin.

Thisen sen pangngai, hrisel tak chuan chutiang beihna laka humhim turin protective shield ang chi protein coating a nei a. A hmaa kan sawi tawh `PIGA` gene hian he venhimna shield siam nan hian a pui a ni. Chuvangin, `PIGA` gene hi a inthlak danglam chuan he protective shield hi a siam tha thei lo. Tichuan, hetiang hian a thleng ta a ni:

1. Attack and Breakdown: I ``Complement System`` hian heng thisen sen invenna nei lo te hi a rawn beih chuan chu cell te chu awlsam takin an chhe vek a, an chhe vek thin. Thisen sen chhunga ``Hemoglobin`` zawng zawng – chu chu oxygen phurtu protein sen chu thisenah a chhuak vek a ni. Doctor te chuan hei hi ``Free Hemoglobin`` tiin an sawi thin.

2. Faina system a chhiat chuan:A tlangpuiin kan taksa hian ``Haptoglobin'' tih hming pu thil a nei a, chu chuan he free hemoglobin hi a man a, a paih chhuak thin. Mahse PNH-ah chuan thisen sen (red blood cells) a chhe nasa lutuk a, he cleaning team hian an zui thei lo.

3. Nitric Oxide tlakchhamna: Taksain he hemoglobin tam lutuk hi enkawl dan dang a hmang a, chu chu chemical nitric oxide an tih a ni. Mahse hei pawh hi a tawp nghal vat thin. I taksain nitric oxide a neih tlem chuan i pum ruh, esophagus (i kawr atanga i pum thlenga kal tube), leh i hnungzang ruh te chu na tak, na tak tak i nei nghal thei a ni.

4. Anemia: Khawthlang lamah chuan thisen sen (red blood cells) a chhiat chuan a lehlamah chuan ruhro (bone marrow)-ah hian thisen sen thar siam turin pressure nasa tak a awm a. Amaherawhchu, an tihchhiat ruala an siam theih loh chuan taksaa thisen sen (red blood cells) a tlem phah thin. Hei hi anemia kan tih chu a ni .

5. Kal chhiatna: He hemoglobin tam lutuk hian kal a tichhe bawk. PNH vei te hi chronic kidney disease vei theihna chance chu a let ruk zetin an pung niin an sawi .

6. Zun thim: A tawp berah chuan he free hemoglobin hian zun a nghawng bawk. PNH vei tam tak chuan zan emaw zingkar emaw an zun hian zun dum an hmu thei. Hei hi zun chhunga hemoglobin concentration a san avangin zun chu a thim ta a ni.

A awlsam zawngin sawi ila, `PIGA` gene a chhiatna avang hian thisen sen (red blood cells) venhimna (protective shield) chu a bo a, immune system ngeiin a tichhia a, harsatna hrang hrang a siam a ni.

He genetic change hian engtin nge platelets a nghawng?

Platelets hi kan thisena cell chi pawimawh dang a ni. Thisen zam siamin thisen chhuah tihtawp nan an pui thin. PIGA gene-a mutation hian thisen sen siamtu stem cell bakah platelet siamtu stem cell te a nghawng a ni.

Hei hian platelet chhia te chu a tlangpui aia thisen zam a siam tam zawk thin . Chumi awmzia chu thisen zam chu a mamawh lohna hmunah, thisen kalna kawng chhungah a lo awm thei tihna a ni. Hei hi `(Thrombosis)` (Thrombosis) an ti a. Hei hi a hlauhawm hle a, a chhan chu heng thisen zamte hi thluak, thinlung emaw, lung emaw thlenga an kal chuan nunna atana hlauhawm tak a ni thei a ni.

PNH natna lan chhuah dan chu engte nge ni?

PNH tih hming hi symptom pakhat (dark urine) atanga lak chhuah ni mah se, PNH vei tam tak chuan doctor an pan hmasa ber a, a chhan chu an hah lutuk a, chu chuan an nitin hnathawh a ti harsa hle a ni Hei hi presenting symptom awm tam ber a ni.

A lan chhuah dan dangte chu:

• Thâwk harsa (`(Dyspnea)`) .
• Kidney lama harsatna (e.g., zun tlem, ke hring) .
• Chaw ei harsa (Dysphagia) .
• Chaw ei kawng (Esophageal Spasms) a awm
• Pum natna
• Hnung lam natna
• Mipa mipat hmeichhiatna lama harsatna (`(Erectile Dysfunction)`) .

Heng symptoms pakhat emaw a aia tam emaw i neih chuan, a bik takin zun rawng inthlak danglamna nen hian doctor thurawn lak ngei ngei a tha ber.

Doctor-te hian engtin nge PNH hi an hriat theih? (Diagnosis) tih a ni.

Doctor i hmuh hunah i natna lan chhuah dan an zawt ang che a, an enfiah ang che. Tichuan, PNH i vei nia an rinhlelh chuan test engemaw zat an nei thei a, chu chu an finfiah thei a ni.

Chung zinga langsar zualte chu:

• Complete Blood Count (CBC w/diff): Hei hian thisen lama harsatna dang, thisen tlakchhamna leh platelet count hniam (thrombocytopenia) te a enfiah thin.
• Basic Metabolic Panel (BMP): Hei hian kal hnathawh dan leh kal natna khirh tak lanchhuah dan a enfiah thin.
• Urinalysis: Hei hian zun chhunga hemoglobin awm (Hemoglobinuria) leh iron tam lutuk (Hemosiderosis) te ang chi thil a hmu thei a ni.
• Reticulocyte Count: Reticulocyte hi thisen sen puitling lo a ni. Chungte chu ruhro hian thisen sen hrisel tak tak a siam chhuak em tih hriat nan chhiar a ni.
• Haptoglobin Test: He protein hian thisen sen chhe tawh atanga bawlhhlawh paih chhuahna hna a thawk a ni. Level hniam chuan thisen sen (red blood cells) a chhe lutuk tihna a ni thei.
• Lactate Dehydrogenase (LDH) test: LDH hi thisen sen chhunga enzyme awm a ni. Thisenah hei hi a tam chuan thisen sen (red blood cells) a tichhia tih a tilang a ni.
• Liver Function Test: He test hian thisen sen (red blood cells) a chhiat chuan bilirubin level a teh thin.

Heng test result atang hian doctor hian PNH chungchangah rinhlelhna dang a neih chuan thisen test bik ``Flow Cytometry'' tih a order thei a ni.Hei hi PNH nei thisen sen (red blood cells) te dik taka hriat theihna a ni.

PNH enkawl dan tur chu engte nge ni?

Tun hma chuan PNH hi natna hlauhawm tak leh enkawl harsa takah an ngai a. Chutih lai chuan a enkawlna ber chu thisen tlakchhamna (anemia) enkawlna atana thisen pek fo hi a ni. Mahse, PNH vei te hi an hmuhchhuah hnuah kum 10 atanga kum 22 chhung an dam tlangpui. PNH tihdam vek theihna awmchhun chu allogeneic stem cell transplant neih a ni . Tin, chu chu mi zawng zawng tih theih a ni lo.

Mahse, tunah chuan dinhmun a danglam ta hle mai!

Vanneihthlak takin tunah chuan PNH tan hian damdawi tha tak tak, targeted therapies a awm ta. Chungte chu complement inhibitor an ti a.Heng damdawi te hian an tih chu ``Complement System`` hian thisen sen (red blood cells) a beih leh a tihchhiat loh nan a titawp a ni.

Zirna hrang hrangah chuan PNH vei heng enkawlna thar dawng te hian PNH nei lo mi ang bawkin dam chhung pangngai an hmang thei tih hmuhchhuah a ni. Hei hi hlawhtlinna ropui tak a ni tak zet!

Enkawlna hian side effect a nei em?

Ni e, heng `(Complement Inhibitors)` damdawi te hian side effect engemaw zat a nei thei. Entirnan, mi thenkhat chuan lu na leh luak chhuak ang chi thil an nei thei. Mahse, thu lawmawm tak chu heng side effect tam zawk hi enkawl tan atanga ni engemaw zat chhungin a reh thin. Hemi chungchang hi i doctor chuan a hrilh belh ang che.

PNH hi ven theih a ni em? Tihdam theih a ni em?

PNH hi genetic mutation avanga lo awm a nih avangin ven dan tur a awm lo.

Enkawlna lamah chuan ``Complement Inhibitor`` damdawi thar hian PNH avanga thisen sen leh platelet tihchhiatna chu a titawp thei a ni. Chumi awmzia chu natna chu thunun theih a ni tihna a ni. PNH vei tam tak chuan thisen tlakchhamna ang chi natna avang hian enkawlna dang an mamawh thei a ni. Sawi tawh ang khan ``Stem Cell Transplant`` tih theih ni mahse, enkawlna buaithlak tak a ni. Tuna damdawi hman mek hmang hian natna hi uluk taka enkawl theih a ni a, miten nun pangngai an hmang thei a ni.

PNH vei angin engtin nge i in enkawl?

Enkawlna nen pawh PNH i neih chuan thisen zam lo turin hma i lak a pawimawh hle. Entir nan:

• PNH vei mi pakhatin operation a neih a ngai a nih chuan , a tih laiin thisen zam leh thisen chhuah tam lutuk a awm thei. Chuvang chuan Doctor ten he thil hi an fimkhur zual hle a ni.
• Naupai hian PNH vei hmeichhiate tan hriselna a tichhe a, anmahni tan pawh leh an nau piang lai tan pawh a hlauhawm hle. Chuvangin naupai hma leh naupai chhung zawnga specialist enkawlna hnuaia awm hi a pawimawh hle.

I doctor chuan i nunphung i tihdanglam tur te, i ngaihven tur thilte ( attention , hei hi Sanskrit/Malayalam a ni a, ka pumpelh tur a ni. Let me rephrase: i nunphung inthlak danglamna i siam tur te, i ngaihven tur thil te.) i nunphung i tihdanglam tur te, i ngaihven tur thil te. Chu thurawn chu zawm a pawimawh hle.

A tawp berah chuan a pawimawh ber (Take-Home Message) .

PNH, a nih loh leh ``Paroxysmal Nocturnal Hemoglobinuria'' hi thisen lama natna awm lo mahse na tak a ni thei. Kum 20 vel kalta khan he natna vei te hian enkawlna tur an nei tlem hle a, hmalam hun pawh a chiang lo hle.

Mahse vawiinah chuan kan dinhmun a danglam ta! Enkawlna thar, tunlai tak takte avang hian PNH vei te hian an thisen cell te chu an humhim thei a, harsatna lian tham tak tak an pumpelh thei a, nun famkim leh hrisel takin an nung thei bawk.

PNH i vei tih hmuhchhuah a nih chuan hlauthawng suh. I doctor chuan i tana tha ber tur enkawlna kawng hrang hrang a hrilhfiah ang che. Enkawlna hian PNH hi a tidam kim lo nain, natna avanga hriselna lama harsatna lian tham lo thleng tur venna atan a pui thei a, nun hrisel tak i neih theih nan a pui thei bawk. A pawimawh ber chu natna lanchhuahna i neih chuan a rang thei ang bera damdawi lam pan a, a tih ang chiaha enkawl dan zawm hi a ni.

` PNH, Paroxysmal Nocturnal Hemoglobinuria, Thisen natna, Hemoglobinuria, thisen tlakchhamna, Thisen zam, Kal natna