AL Amyloidosis hi eng nge ni? He natna hlauhawm tak hi han sawi ila!

He natna `AL Amyloidosis` tih hi i hre tawh em? A ni lo mai thei, a dik em? Hei hi a takah chuan a tlem deuh a, mi zawng zawng nghawng thei natna a ni lo tihna a ni. Mahse, a lo thleng a nih chuan a pawi deuh thei. A awlsam zawngin kan taksaa kan neih ``Plasma cells`` tih cell chi khat inthlak danglamna a ni a, an siam protein thenkhat chu control theih lohvin kan taksa peng hrang hrangah an dah a, an tichhia a ni. Machine pakhatin hna a thawk tha a, rang takin a part dik lo a siam tan ang mai a ni. Hemi chungchang hi tlem tal sawi ila , a chhan chu hei hi hriat chian a pawimawh hle.

`AL Amyloidosis` tih hi eng nge ni tak tak?

He `AL amyloidosis` hi natna pawl lian zawk chi khat `Amyloidosis` an tih a ni. `Amyloidosis` hi kan ruhroa `plasma cells`-a inthlak danglamna, a nih loh leh `mutation` a awm hian natna hmuh tur awm lo tak a ni. Heng plasma cell danglam tak takte hian protein chi danglam tak an siam chhuak thin. Chûng protein-te chu thread ball inzawm khawm ang maiin an inzawm khawm a, kan taksa peng pawimawh tak tak leh kan taksa peng hrang hrangah an dah khawm a ni.

AL amyloidosis ah chuan hetiang hian protein chi khat a buai thin chu light chain protein a ni. Heng light chain proteins te hi kan taksain natna dona atana a hman antibody thenkhat a ni. Heng antibodies te hi a hmaa ka sawi tawh plasma cell ang chiah hian siam a ni.

A tlangpuiin, AL amyloidosis hian kan thinlung leh/ emaw kal (kidney) a nghawng tam ber . Mahse, a chang chuan pum, intestine, nerve leh vun te a nghawng thei a ni.

A tha ber chu he natna hi hriat hmasak a, enkawl vat a nih chuan , a chang chuan natna khirh tak angin enkawl theih a ni. Mahse, enkawl loh a nih chuan nunna atana hlauhawm tak tak leh thihna thlengin a thlen thei a ni. Chuvangin hei hi ngaihthah tur a ni lo.

He hminga hawrawp pahnih `AL` hian eng nge a awmzia?

Amyloidosis hi chi hrang hrang a awm a. A chi tin hi natna thlentu protein pangngai lo hming chawia vuah a ni. AL amyloidosis-ah chuan A letter hian amyloidosis a entir a, L letter hian natna thlentu protein light chain an tih a entir bawk. Amyloidosis chi dang hriat lar tak takte chu AA amyloidosis (serum amyloid A protein avanga lo awm) leh ATTR amyloidosis (transthyretin avanga lo awm) te an ni.

Engtin nge `AL amyloidosis` hian ka taksa a nghawng?

A awlsam zawngin AL amyloidosis hi plasma cell natna a ni. Plasma cells hi kan immune system-ah hian a tel ve a ni. An hna ber chu hri kai dona atana antibody siam hi a ni. Kan taksaah hian natna chi hrang hrang do turin antibody chi hrang hrang siamtu plasma cell clone sang tam tak a awm tih han ngaihtuah teh. Plasma cell pakhat chuan a inthen darh a, cell tam tak a siam belh leh a ni.

‘Plasma cell’ kaihhnawih natnaah chuan heng ‘plasma cell’ pawl khat chu thunun theih lohvin an inthen a, ‘antibody’ chi khat tam tak an siam chhuak thin.

AL amyloidosis-ah chuan antibodies siam a nih chuan protein chain pahnih heavy chain leh light chain an tih chu siam a ni. Heta thil thleng hi plasma cells te hian light chain proteins an siam tam lutuk hi a ni. Heng light chain te hi an inzawm sual a, an inzawm khawm a ni. Heng protein inzawmkhawm te hi amyloid fibrils an ti a. Heng fibrils te hi kan taksa peng hrang hrangah a awm ta a ni. Chutah chuan chu taksa peng hrang hrangte chu chhiatna nasa tak a thleng tan a , a châng chuan nunna atana hlauhawm thei khawpa chhiatna a thleng ṭan a ni.

He natna hi tuin nge vei tam ber?

Amyloidosis hi natna hlauhawm tak a ni. United States-ah chuan maktaduai khatah mi 9 atanga 14 inkar velin a vei nia chhut a ni a, khawvel hmun dangah chuan mi maktaduai khatah mi 5 atanga 12 inkar velin a vei nia chhut a ni. AL amyloidosis hi hmeichhia aiin mipaah ​​a tam zawk deuh. Kum 60 chunglam pawh a vei tlangpui a, kum 64 vel a ni.

`AL amyloidosis` natna lan chhuah dan chu engte nge ni?

`AL amyloidosis` hian kan taksa peng hrang hrang, lu atanga ke thlengin a nghawng thei a, ke leh ke ruhte pawh a nghawng thei a ni. Vawi tam tak chu he natna lan chhuah dan hi natna dang, natna khirh lo zawk chhinchhiahna nen a inang thei a ni. Tin, a symptoms te pawh a lang zawi zawi hle bawk . Chuvangin i taksaah inthlak danglamna i hmu nghal lo mai thei.

Lu leh kawng nena inzawm natna lan chhuah dan:

• I thawh chuan i lu a na emaw, i lu a na (`lightheadedness`) emaw i ti em?
• I mit vel emaw, i mitmengah emaw, purple rawng hring i nei em?
• I lei chu a tlangpui aia lian zawkin i hria em ?

Kut leh ke nena inzawm natna lan chhuah dan:

Kut hian heng mizia te hi a nei thei a ni:

• I kut ah hian tingling, burning, prickling sensation i nei em? Hengte hi ``Peripheral Neuropathy`` tih natna lanchhuahna a ni thei.
• I kut zungtang ah hian tingling leh numbness i nei em? Hengte hi ``Carpal Tunnel Syndrome`` chhinchhiahna a ni thei.

Ke leh kea natna lan chhuah dan chu:

• I ke nge i ke a hring em ?
• I ke hi nunna nei lo niin i hria em ?

Chu bâkah, i hliam awlsam , thisen chhuak awlsam, emaw, vun rawng sen hring (purple discoloration of the skin where there are wrinkles) i nei tih i hmu thei bawk.

Lung leh lung lama harsatna nei tih hriat theihna symptoms:

A hnuaia symptoms te hi natna dang symptoms a ni thei bawk a, mahse fimkhur hle ang che:

• Heart palpitations: Thinlung a na a, a tlan chak ang maia hriatna .
• Thâwk lak tawi (dyspnea): Kawchhung a thawmhnaw, thawk thûk thei lo ang maia inhriatna a ni.
• Chest pain : Chhung khawi hmunah pawh a na. He natna hi a na hle thei a, a dull thei a, a lo kal leh chhuak thei bawk. Chest na hi heart attack chhinchhiahna a ni thei bawk a, chuvangin i rilru natna minute nga aia rei i neih a, chawlh leh damdawi ei avanga a reh loh chuan 911 call nghal la, damdawi inah mi pakhatin hruai rawh.
• Chauhna : Nitin hna pawh thawk thei lo khawpa chau anga inhriatna, i taksain nunna a nei tawh lo ang maia inhriatna.

Pum emaw, intestinal emaw lama harsatna nei tih hriat theihna symptoms:

AL amyloidosis hian i ei leh in a nghawng thei bawk. Symptoms i nei thei a, chungte chu:

• Chaw ei chak lohna : Mi tin ei duhna hi a chang chuan a inthlak danglam thin. Mahse chhan awm lovin ni engemaw zat ei duhna i hloh a nih chuan AL amyloidosis nena inzawm intestinal problem chhinchhiahna a ni thei.
• Bloating emaw gas tam lutuk : Bloating leh gas hi a awm fo thin a, mahse a chang chuan zahthlak tak a ni. Mahse, persistent gas i neih chuan AL amyloidosis nena inzawm, pum chhunga harsatna i neihna chhinchhiahna a ni thei.
• Constipation : Constipation chu kar khatah vawi thum aia tlem lo chhuah hi a ni. Kar thum aia rei constipation i neih chuan doctor nen inbiakna neihpui tur harsatna a ni.
• Diarrhea : Tui hmanga bawlhhlawh awm. Diarrhea a awm reng chuan doctor hnenah pawh i hrilh tur a ni.

Kidney emaw bladder emaw lama harsatna nei tih hriat theihna symptoms:

I zun lan dan emaw, zun i mamawh zat emaw a danglam tih i hmu thei. Heng symptoms te hi a hnuaia mi ang hian a ni thei:

• I zun ah hian a tlangpui aia tam bubble i hmu em?
• I zun a tlem zawk em , a nih loh leh zanah i zun tur chuan i tho a ngai em ?

Eng thilin nge AL amyloidosis hi a thlen?

Hemi chungchang hi tlem kan sawi tawh a. `AL amyloidosis` hi `plasma cells` atanga lo awm a ni a, chu chuan `antibodies`, proteins `heavy chains` leh `light chains` an tih te chu a siam a, chu chu `light chain` proteins te chu a tam lutuk chuan a siam a ni. Heng `light chains` te hi an inzawm sual a, an inzawm khawm a, `amyloid fibrils` an siam a, chu chu kan taksa peng hrang hrangah a lo awm ta a ni. Chutah chuan buaina chu a intan ta a ni.

Doctor-te hian engtin nge AL amyloidosis hi an hriat theih?

Doctor-te chuan AL amyloidosis hmuhchhuah nan test eng emaw zat an hmang a. Test pawimawh ber chu natnain a nghawng nia ngaih tissue emaw organ emaw sample tlemte lak a ni. Hei hi biopsy an ti a. Biopsies hi a hnuaia mi ang hian kan thliar hrang thei ang:

• Bone marrow biopsy : Hei hi ruh chhunga ruhro sample tlemte lak a ni.
• Kidney biopsy : Kidney tissue te te tlemte lak theih a ni.
• Heart biopsy : Heart muscle te te tlemte lak theih a ni.
• Fat pad biopsy : Pum atanga thau te tak te lakchhuah a ni. Hei hi test awlsam tak a ni.

Test dang pawh a awm bawk

I doctor chuan i taksa peng hrang hrangte a thawk tha em tih hriat nan test dang a ti thei bawk. Chûng zîngah chuan:

• Thisen test : Hengte hi i kal, thinlung leh thin hnathawh dan enfiah nan leh i thisenah light chain awm zat enfiah nan an ti thin.
• Urine test : Hei hi a tlangpuiin darkar 24 chhunga zun lakkhawmna test a ni. In lamah i zun i la khawm a, i doctor hnenah sample i rawn keng thin. He test hian i kal (kidney) te chu amyloidosis in a nghawng em tih a enfiah thin.
• Electrocardiogram (ECG): ECG hian thinlunga electric hnathawh dan a teh a.
• Echocardiogram : A chang chuan ultrasound of the heart an ti a, hei hian high-frequency sound wave hmangin thinlung chetna a teh thin.
• Cardiac MRI (Cardiac Magnetic Resonance Imaging) : He test hian thinlung thlalak chiang tak, chipchiar tak tak a la thei a ni.

AL amyloidosis hi engtin nge enkawl a nih?

Doctor-te chuan AL amyloidosis hi i natna lan chhuah dan tihtlem nan, taksa peng chhiatna control nan leh amyloid protein pangngai lo awm zat tihtlem emaw, tihtawp emaw nan an enkawl thin.

Doctor-te chuan AL amyloidosis hi chemotherapy, immunotherapy, steroid hmangin an enkawl thei a ni. Mi tam zawk chu chemotherapy damdawi pakhat emaw pahnih emaw pek an ni a, a tam zawkah chuan steroid nen an inkawp thin. Heng damdawi te hian an thawk dun a, light chain protein siamtu plasma cell te chu an tichhia a ni.

Pawimawh tak chu heng damdawi te hian AL amyloidosis natna hi a titawp thei a, a tikhawtlai thei a, mahse i taksa peng hrang hranga amyloid fibrils lo awm tawh chu a paih chhuak thei lo. Enkawlna a tan hnuah chuan nangmah ngei pawhin heng protein pangngai lote hi zawi zawiin a paih chhuak thei a ni. Researcher te chuan heng fibrils te hi paih chhuak thei tur monoclonal antibodies thar an zawng mek bawk.

I doctor chuan ``bone marrow transplant`` emaw ``stem cell transplant emaw atanga hlawkna i hmuh theih dan tur chungchang pawh a sawipui ang che.`` Hengte hi enkawlna buaithlak zawk ni mahse mi thenkhat tan chuan a hlawhtling hle thei a ni.

`AL amyloidosis` lo awm tur hi ven theih a ni em?

Vanduaithlak takin, ni lo. AL amyloidosis emaw amyloidosis chi dang emaw ven dan tur a awm lo. AL amyloidosis hi plasma cell pakhat chu a pangngai lo a, thunun theih loha a inthen tan chuan a lo awm thin. Hei hi kan thunun theih thil a ni lo.

`AL amyloidosis` nen hian eng chen nge i dam theih ang?

Tunah chuan Doctor-te chuan AL amyloidosis vei te chu dam rei zawk turin an pui mek a ni. Mi thenkhat tan chuan AL amyloidosis hi hun rei tak, a nih loh leh damdawi hmanga enkawl theih a ni. Mahse, hei hi natna khirh tak a nih avangin nunna atana hlauhawm damdawi lam harsatna a thlen thei tih hriat reng a pawimawh.

AL amyloidosis hi natna hmuh tur awm lo tak a nih avangin he natna vei hi eng chen nge a dam theih ang tih hi doctor tan chuan hriat chian a harsa hle. AL amyloidosis i neih chuan i doctor hnenah enkawl hnuah i prognosis zawt rawh. I doctor chuan i beisei tur chungchangah information tha ber ber a pe thei che a ni.

Engtin nge ka in enkawl ang?

AL amyloidosis i neih chuan i doctor chuan i natna lan chhuah dan enkawl leh natna zual zel tihtawp emaw tihtawp emaw chu a ngaihtuah ber ang. Damdawi i ei chhunzawm zel a ngai mai thei a, chu enkawlnate chuan nghawng ṭha lo a nei thei bawk. I in enkawl theih dan pakhat chu i enkawlnate nghawng tha lo leh enkawl dan tur hriat hi a ni. Thurawn dang thenkhat chu hetiang hi a ni:

• Enkawlna thenkhat chuan i ei duhna a tichhe thei.Chutiang a nih chuan i doctor hnenah enkawlna hun chhunga chak taka awm theihna tura puitu tur ei leh in hrisel chungchang zawt rawh. Ei leh in inthlau tak ei hi a pawimawh hle.
• Chawlh hahdam tam tak neih tum ang che . Hetiang natna kan do dawn hian taksa tan chawlh hahdam a pawimawh hle.
• Exercise hi i rilru a hlim theihna tur leh taksa rihna hrisel tak neih theihna tura puitu tha tak a ni. Mahse, i taksaah harsatna nasa tak siam lo tur exercise chungchangah doctor hnenah thurawn dil rawh.
• AL amyloidosis hi natna hmuh tur awm lo tak a nih avangin mahni chauha awm leh inhlat takin i awm thei . Mi dangte chuan natna hi an hrethiam lo mai thei. AL amyloidosis vei te tana support group emaw programme emaw chungchang i doctor zawt rawh. Chutiang mite nena inbiakna chu chakna petu ropui tak a ni thei.

A tawp berah chuan thu pawimawh tlemte (Take-Home Message) .

AL amyloidosis (Amyloid light chain or primary amyloidosis) hi kan taksa peng leh taksa peng hrang hranga light chain proteins pangngai lo (abnormal light chain proteins) a awm vang a ni. It can be a very serious disease , a chang chuan natna khirh tak anga enkawl a ni a, mahse nunna atana hlauhawm damdawi lam harsatna a thlen thei bawk.

Mahse lungngai suh, doctor-te chuan AL amyloidosis vei te chu rei zawk leh dam zawk an dam theih nan kawng thar an zawng mek a ni. AL amyloidosis i neih chuan i doctor hnenah clinical trial thar leh natna enkawlna atana research chungchang zawt la. Hriat hmasak leh enkawl hi a pawimawh hle. Tin, positive taka awm reng a, doctor thurawn zawm a pawimawh bawk.

` AL amyloidosis, primary amyloidosis, plasma cell, light chain protein, amyloid fibrils, kal natna, thinlung natna