Langerhans Cell Histiocytosis (LCH) hi eng nge ni? I naupang tê chu hlauhawmah a awm em?

Langerhans Cell Histiocytosis, a nih loh leh a tawi zawnga LCH kan tih hi i hre tawh em? A hming hi a buaithlak deuhin a hlauhawm deuhin a ri mai thei. Mahse a nihna takah chuan hei hi natna hmuh tur awm lo tak a ni a, kan naupang te, a bik takin nau piang thar leh naupang kum 15 hnuai lam a tuar ber a ni. Chuvangin lungngai suh, a awlsam zawngin sawi ila, Sinhala tawngin i hriatthiam theih em em, chipchiar takin, i thian hnai tak i biak ang maiin.

(LCH) hi eng nge ni tak tak?

A awlsam zawngin (LCH) hi naupang taksaah Langerhans cell tam lutuk, kan immune system-a special cell chi khat a awm tam lutuk avanga lo awm thin a ni. Heng Langerhans cells te hi a takah chuan thisen var chi khat an ni. An hnathawh ber chu kan taksaa hrik lutte do a, natna laka min humhim hi a ni.

Heng cell te hi a tlangpuiin kan taksa pum puiah a awm a, a bik takin vun, lung, lymph node, ruhro, ril, leh thin ah te a awm thin. Mahse, (LCH)-ah chuan heng Langerhans cell te hi hmun khat emaw a aia tam emaw ah an pungkhawm nasa hle . Chutiang a nih chuan heng taksa peng hrang hrangte hi a chhe thei a, hliam a lo awm thei bawk.

Natna kal dan hi mi hrang hrangah a inang lo thei. Naupang \henkhat chu a \ul anga enkawl a nih chuan natna a\angin an dam kim vek \hin. Mak tak maiin, a chang chuan, a bik takin vun chauh a nghawng a nih chuan engmah enkawl ngai lovin a dam thei a ni. Mahse, he natna (LCH) hian naupang ruhro, ruhro emaw, thin (liver) ang chi taksa peng pawimawh tak takte a nghawng a nih chuan enkawlna nasa zawk a ngai mai thei.

(LCH) hi cancer a ni em?

Hei hi mi tam takin zawhna an neih a ni. Dik tak chuan zirchiangtu tam zawk chuan LCH hi cancer natna, chu chu neoplasm tihna a ni. Mahse, tunah chuan scientist thenkhat chuan inflammatory disease ah an ngai ta a ni. Mahse, oncologist te hi cancer leh thisen natna lama tui bik doctor an ni. A châng chuan cancer enkawlna, Chemotherapy ang chite pawh hi a natna enkawl nân an hmang bawk.

He (LCH) natna hian tute nge a nghawng ber?

LCH hi nausen piang thar leh naupang kum 1 leh 15 inkarah a tam ber a, puitlingah chuan LCH hi a tlem hle a, mahse thil theih loh a ni lo.

Hei hi engtiang chiahin nge a hluar?

Statistics atanga a lan dan chuan nau piang thar maktaduai khat zinga pakhat emaw pahnih emaw chu kum tin LCH hian a vei thin. Tin, naupang kum 15 hnuai lam maktaduai zinga panga velin a nghawng bawk. Chuvangin hei hi thil awm lo tak a ni tih i hmu thei.

(LCH) natna lan chhuah dan chu engte nge ni?

LCH natna lan chhuah dan hi mi hrang hrangah a inang lo hle. Taksa hmun khat chauh a nghawng thei a, hmun tam tak a nghawng thei bawk. Chuvangin, a symptoms chu naupang taksa peng eng ber nge a nghawng tih ah a innghat a ni.

Ruh a nghawng chuan:

LCH vei naupang 80% vel chuan an ruh pakhat emaw a aia tam emaw ah hliam an nei thin. Eng thilin nge hetiang hi a thlen theih?

• Luruh, mit vel ruh, beng ruh, hmui ruh, ke ruh, ruhro, hip emaw, ruh emaw ang chi hmunah hian a hring emaw, a hring emaw a lo awm thei a ni. He hliam hi a na thei a, a reh thei bawk.
• Lu na.
• Kawng emaw hnungzang emaw a na.
• Fractures a awm.
• Kal harsat.
• Limping a ni.

Vun chungah chuan:

Rashes hi vun chhinchhiahna (LCH) anga ngaih a ni tlangpui.

• Nausen naupangteah chuan lu vunah cradle cap ang maia hring a lo lang thei.
• Naupang kum upa zawk leh puitlingah chuan flaky rash a siam thei a, chu chu dandruff ang mai a ni.
• Heng rashes te hi taksa peng dang (groin, arms, armpits, stomach, back, chest) ah te pawh a lo lang thei bawk. An na thei a, an hrual a, an khauh thei bawk.
• Naupang thenkhatah chuan oozing blisters an nei thei bawk.
• Nail rawng danglam, hardening, peeling ang chi thil pawh a thleng thei bawk.

Kawngkhar chungchangah chuan:

Ka chhunga symptom hmuh theih te chu:

• Ha hring emaw ha hloh emaw.
• Ha a chhuah.
• Gums a hring chhuak.
• Ka hmuiah emaw, leiah emaw, hmui chhungah emaw, hmui chung lamah emaw hliam.

Liver emaw Spleen emaw nena inzawm:

Heng taksa peng hrang hrangte hi a nghawng a nih chuan a lan chhuah dan te chu:

• Liver leh/ emaw spleen lian lutuk avanga pum chhunga natna.
• Vun sen leh mit var (jaundice).
• Itching a ni.
• Chau.
• Bruising leh/ emaw thisen chhuah awlsam.

Bone marrow a nghawng chuan:

Bone marrow hi thisen cell siamna hmun a ni. A nghawng a nih chuan:

• Thisen sen (red blood cells) tlahniam vanga thisen tlakchhamna, vun dum, hahna, leh ei duh lohna te.
• Thisen var tlakchham vanga natna hrik kai fo leh khawsik.
• Platelet tlahniam avanga hliam awlsam leh/ emaw thisen chhuak.

Endocrine System (Endocrine System - Hormone) te hi a awm a, a chhuahna tur hmun leh a hmanna tur a awm lo.

LCH hian naupang endocrine system a nghawng thei a, chu chu hormone siamna hmun a ni a, pituitary gland leh thyroid gland te hi a ni.

• Pituitary gland a nghawng chuan: tuihal lutuk (polydipsia) leh zun tam lutuk (diabetes insipidus an ti bawk), thang chak lo, puitlin hma emaw, tlai emaw, leh taksa rihna sang.
• Thyroid gland a nghawng chuan: thyroid gland a hring, thyroid hormone level hniam (hypothyroidism), leh thawk harsa tihna te.

Beng chungchangah chuan:

• Beng natna a awm fo thin.
• Beng atanga tui chhuak.
• A sen hring.
• Itchy rash a awm.
• Beng na.
• Hriatna hloh.

Mit chungchangah chuan:

• Mit bulging tak tak.
• Mit chungah a hring chhuak.
• Mit hmuhna lama harsatna a awm.

Lymph Nodes te chu: 1.1.

• Kawng, kawr, leh/ emaw, ke ruh (groin)-a lymph node hring leh na tak tak.

Central Nervous System: A rilru a hah lutuk chuan a rilru a buai em em a.

Chumi awmzia chu thluak leh/ emaw, ruhro (spinal cord) a nghawng tihna a ni.

• Lu na.
• Luak chhuak thei.
• Luak chhuak.
• Tuihal lutuk.
• Zun chhuah fo thin.
• Kal harsat.
• Balance hloh.
• Taksa che vel control theih lohna (ataxia).
• Thusawi harsa leh hmuh harsa.
• Seizures a awm thin.
• Thiltih leh/ emaw hriatrengna inthlak danglamna.

Chuap:

Pulmonary LCH, lung a tichhe thei hi puitlingah a tam ber. Meizial zu thinte hi an hlauhawm zual hle.

• A rilru a na.
• Dry cough a ni.
• Thâwk harsa.
• Thisen a khuh chhuak.
• Lung chak lohna (pneumothorax) a awm.

Gastrointestinal Tract-a awmte chu:

Naupang pum, small intestine, leh/ emaw large intestine a nghawng chuan:

• Pum natna.
• Luak chhuak.
• Kawthalo.
• Thisen chhuak chu stool ah a awm.
• Ei leh in tha lo avanga thang chak lohna.

Pawimawh: Heng symptoms te hi LCH chauh ni lovin, condition dang engzat pawhin a awm thei. Chuvangin i fain heng symptoms te hi a nei a nih chuan a natna chiang taka a hriat theih nan damdawi lam thurawn lak nghal a pawimawh hle.

(LCH) awm chhante chu engte nge ni?

LCH vei zinga a chanve vel chauh hian BRAF an tih gene-ah somatic mutation an nei a ni. Somatic mutations chu naupai hnua cell thenkhata inthlak danglamna a ni. Nu leh pa atanga rochun an ni lo va, embryo lo pian chhuah lai hian random takin an lo awm thin.

(BRAF) gene hian cell than leh thanlenna control tu protein a siam chhuak a. A tlangpuiin he protein hi chemical signal angin on leh off theih a ni. Mahse, he gene-ah hian mutation a awm chuan protein chu "on" state-ah a awm reng a ni. Hei hian (LCH) cell te chu a ti thang lian a, a inthen nasa lutuk bawk. Hei hian tissue a tichhia a, tumor a siam thin.

Scientist-te pawhin gene dang engemaw zat mutations hian natna hi a thlen thei tih an hmuchhuak bawk. Entirnan, genes (MAP2K1), (RAS), leh (ARAF) te hi a ni. Zirchiangtu thenkhat chuan environment toxins leh viral infection te pawhin he natna hi a thlen thei niin an ngai.

(LCH) tana risk factors te chu engte nge ni?

Thil thenkhat chuan i fain LCH a vei theihna a tipung thei a ni. Chung zingah chuan:

• Chhungkaw history nei LCH nei.
• Hispanic hnam neih (hei hi kan ram nen hian a inzawm tawk lo nain, source hrang hrangah tarlan a ni).
• Meizial zuk (a bik takin puitling lung cancer (LCH) nei tan).
• Naupai laiin chemical thenkhat nena inzawmna.
• Hnathawhna hmuna thir, granite emaw thing leivut emaw nena inzawmna.
• Nau piang thar hun chhunga hri kai.
• Naupan laia vaccine pek tura rawtna pek loh.

(LCH) avanga harsatna awm thei te chu engte nge ni?

LCH vei naupang 50% vel chuan he natna avang hian harsatna hrang hrang an nei thin. Entir nan:

• Scarring a awm.
• Growth tihkhawtlai a ni.
• Musculoskeletal lama harsatna nei.
• Zunthlum (Diabetes insipidus) ang chi natna.
• Hormone inthlauhna (hormone inthlauhna) a awm.
• Hriatna lama harsatna nei.
• Rilru hriselna lama harsatna (depression, lungkhamna ang chi).
• Ruh leh lung lama harsatna nei.
• Liver cirrhosis a awm thin.
• Cancer pahnihna (e.g. leukemia, lymphoma, Ewing sarcoma) te pawh a awm thei.

Engtin nge LCH hi hmuhchhuah a nih?

I fa doctor chuan i fa medical history a zawt hmasa ang a, physical exam a ti ang. Tichuan, i fain a natna lan chhuah dan azirin test engemaw zat an order ang. Heng test result a zirin i fa chu pediatric hematologist/oncologist hnenah refer theih a ni a, ani chuan i fa enkawlna leh enkawlna chu a coordinate ang.

Diagnosis atan eng test nge tih thin?

LCH hian taksa peng hrang hrang a nghawng theih avangin natna dik tak hriat theih nan test eng emaw zat neih a ngai a ni.

• Thisen test dan: 1.1.
• Complete blood count (CBC): Hei hian naupang thisen sen (red blood cell), thisen var leh platelet level te a enfiah thin.
• Thisen chemistry test: Hengte hian taksa peng leh tissue atanga thil thenkhat chhuah zat a enfiah thin.
• Liver function test: Hengte hian liver atanga thil chhuak zat a enfiah thin.

• Zun test dan: 1.1.
• Urinalysis: Naupang zun ah hian thisen sen, thisen var, protein, leh sugar te a awm leh awm loh enfiah thin a ni.
• Water deprivation test: Hei hian naupang zun engzat nge a zun tih leh tui pek loh chuan zun a concentrate em tih test a ni.

• Biopsies te chu:
• Biopsy: Doctor chuan tissue sample a la a, pathologist chuan microscope hmangin LCH cells a awm leh awm loh a en a.
• Bone marrow aspiration leh biopsy: Hollow needle hmangin naupang hip ruh atanga ruhro, thisen leh ruh te tak te te chu lakchhuah a ni. Hei hi pathologist pawhin a enfiah thin.

• Genetic testing neih dan tur: 1.1.
• Thisen emaw tissue sample emaw hmangin (BRAF) gene a inthlak danglam leh awm loh enfiah thin a ni.

• Imaging test hrang hrang:
• X-ray: Taksa ruh leh taksa peng hrang hrang thlalak lak a ni. A châng chuan taksa pum pui ruh (skeletal survey) hmangin thil dik lo zawngin an enfiah ṭhin.
• Bone scan: Radioactive substance chu vein pakhatah inject a ni a, scanner hmangin ruh chhunga thil awm dan pangngai lo awmte chu an zawng thin. Hei hi hman tam a ni tawh lo.
• CT scan (Computed tomography - CT scan): Naupang chu tui bik in tur emaw, vein-ah inject emaw a ni a, taksa chhung lam angle hrang hrang atanga thlalak chipchiar tak tak lak a ni.
• Positron emission tomography (PET scan): Radioactive sugar (glucose) tlem te te chu vein pakhatah an inject a, chu scanner chu taksa chhungah an kal kual a, chu chu sugar hmanna hmun thlalak a la a ni. He scan-ah hian natna cell te chu a eng chhuakin a lang.
• MRI scan (Magnetic resonance imaging - MRI scan): Thil pakhat gadolinium an tih chu vein-ah inject a ni a, magnet, radio wave leh computer hmangin thlalak chipchiar tak tak a la a. Natna hmunte chu a eng zawkin a lang.
• Ultrasound: High-energy sound wave hmangin taksa chhung lam thlalak a siam a, taksa peng leh tissue atanga back echoe te chu a rawn lang chhuak thin.

LCH hi engtin nge enkawl a nih?

LCH enkawlna chu naupang taksaa LCH cell awmna hmun leh natna hi "low-risk" nge "high-risk" a nih leh nih lohah a innghat a ni.

Risk hniam tak tak taksa peng hrang hrangte chu:

• Vun
• Ruh
• Chuap
• Lymph nodes a awm thin
• Gastrointestinal system a ni
• Pituitary gland a ni
• Thyroid gland a ni
• Thymus a ni

Risk sang tak nei taksa peng hrang hrangte chu:

• Thin
• Spleen (Spleen) a ni
• Bone marrow a ni
• Central nervous system (CNS) atanga lo chhuak a ni.

Doctor-te chuan he natna (LCH) hi "single-system LCH" leh "multi-system LCH" tiin an thliar hrang a ni. Chumi awmzia chu:

• Single-system (LCH): LCH cell hi taksa peng pakhat emaw taksa peng pakhatah chauh a awm a. A chi tlangpui chu LCH a ni a, ruh chauh a nghawng a ni.
• Multisystem (LCH): Organ pahnih emaw a aia tam emaw, taksa pum puiah (LCH) cell a awm a. Hei hi single-system (LCH) aiin a tlem zawk hle.

Thil engemaw takah chuan, a bik takin vun emaw ruh emaw (LCH) chauh a nghawng teah chuan enkawl ngai lovin a reh thei a ni. Chutiang hunah chuan doctor-te chuan natna hi a lo let leh em tih leh a darh leh tawh loh an enfiah ang.

(LCH) Enkawl dan tur: 1.1.

• Steroid therapy: I doctor chuan steroid, prednisone ang chi a hmang thei a, a bik takin vun LCH atan a hmang thei. Hengte hian thisen var (white blood cells) hnathawh a ti tlem a, hei hian LCH cell te pawh a nghawng thei bawk.
• Surgery: LCH tumor leh a chhehvel tissue te chu surgery hmanga lakchhuah theih a ni. Curettage an tih procedure pakhat chu ruh atanga LCH cells te chu spoon ang maia hmanraw (curette) nalh tak hmangin an hrual a ni.
• Chemotherapy: Hei hian cancer cell te chu an thang chak lohna turin damdawi pek a ni. Cell te chu an that emaw, an inthen darh emaw an titawp emaw a ni. Heng damdawi te hi kaa ei a ni a, vein emaw muscle emaw-ah inject a ni a, a chang chuan vunah cream angin hnawih a ni bawk.
• Radiation therapy: High-energy X-ray emaw radiation chi dang emaw hmangin cancer cell te chu an that emaw, an lo thang lian leh a inthen darh loh nan emaw an hmang thin.
• Immunotherapy: Naupangin cancer dona atana a taksa hriselna (immune system) a hman dan a ni. Taksa ngeiin a siam emaw, laboratory-a siam emaw hmanga taksa invenna (natural defense) a tichak a ni.
• Targeted therapy: Cancer cell bikte hriatchhuah leh beih nan damdawi emaw thil dang emaw a hmang thin. Entirnan, damdawi BRAF inhibitor an tih hian cancer cell te chu cell thanna atana mamawh protein te a block a, a that thei a ni. Monoclonal antibodies hi immune system protein lab-a siam a ni.
• Stem cell transplant: Chemotherapy hmanga thisen siamtu cell tihchhiat thlakna tur cell thar thlak.

LCH hi ven theih a ni em?

LCH hi a tam zawk chu ven theih a ni a, a chhan chu genetic mutation avanga lo awm a nih vang a ni. Risk factor thenkhat, chhungkaw history leh ethnicity te hi kan thunun thei lo. Mahse, kan enkawl theih tur thil engemaw zat a awm a:

• Meizial zuk loh tur.
• Naupai laiin chemical tha lo thenkhat ei loh.
• Vaccine pek tur rawt zawng zawng lak vek.

(LCH) hi eng prognosis nge ni?

LCH hmalam hun hi thil engemaw zatah a innghat a ni:

• Taksa system emaw organ engzat nge a nghawng?
• Eng taksa system emaw, eng taksa peng emaw nge a nghawng.
• Natna hian enkawlna a dawng tha em em.

A tlangpuiin naupang single-system (LCH) leh multisystem (LCH) nei, liver, spleen, leh bone marrow tichhe lo chu "low-risk" category-ah an tel thin. Enkawlna a awm chuan he category-a naupangte hian damchhuah theihna chance 100% vel an nei a ni. Mahse, natna hi a lo lang leh thei a,/ emaw, hun rei tak chhunga harsatna dang a thlen thei bawk.

Naupang multisystemic leukemia (LCH) nei, liver, spleen, emaw bone marrow emaw tibuaitu chu "high risk" category-ah an tel a ni.

Engtikah nge ka fa hian doctor a hmuh ang?

I fain a enkawl zawh hnuah pawh doctor chuan i fa chu a en reng a ngai dawn a ni. Hei hi a chhan chu natna lo lang leh theihna a sang hle a ni. Chuvangin i fa chu kum eng emaw zat enfiah a ngai dawn a ni. Heng follow-up test neih chhung hian naupang natna hmuhchhuah a nih laia test tih ang chi, ultrasound, MRI, CT scan, leh PET scan te pawh tih nawn leh a ngai dawn a ni. Doctor chuan i fa i hruai luh tur zat a hrilh ang che.

Ka fa doctor hnenah eng zawhna nge ka zawh ang?

I fa diagnosis chungchangah zawhna tam tak i nei mai thei. Chu chu ziak chhuakin, i doctor appointment lo awm turah i hruai ve mai tur a ni. Zawhna i zawh theih ṭhenkhat chu hetiang hi a ni:

• Langerhans cell histiocytosis hian engtin nge ka fa chu a nghawng ang?
• Ka fa hian enkawlna a mamawh em?
• Ka fa tan hian eng enkawlna kawng nge awm?
• Enkawlna hian eng side effect nge a neih?
• Heng enkawlna hmang hian ka fa hi a dam vek dawn em ni?
• Ka fa damlohna tur hmalam hun chu eng nge ni?
• Support group i recommend theih tur a awm em?

A tawp berah chuan Take-Home Message kan rawn thawn leh ta

Langerhans Cell Histiocytosis (LCH) hi natna hlauhawm tak a ni a, naupangte hi a vei tam ber a ni. I fa hian hetiang hi a tawk a nih chuan rilru natna nasa tak i nei mai thei. I fa tan lungngai leh hlauhna i nei thei. I chhungril lamah chuan i hlauthawng hle mai thei a, mahse pawn lamah chuan chak taka awm i tum a ni mai thei. I rilru put hmang zawng zawng hi a dik vek a ni. Mahse, he zinkawng hi nangmah chauhva i paltlang a ngai lo tih hre reng ang che. I fa medical team chuan i thil tawn chu an hre chiang hle. I kalna apiangah tanpui turin, i fa dinhmun hrethiam tur leh tuar chhuak thei tura chakna pe turin an awm a ni.

I fa chu a hmaa hriatchhuah leh enkawl dan dik chuan hriselna tha zawk a pe thei tih hre reng ang che. Hlau suh, hei hi huaisen takin hmachhawn rawh.

` Langerhans Cell Histiocytosis, LCH, naupang enkawlna, cancer, genetic mutations, symptoms, enkawl dan