Bone Marrow Failure: Hemi chungchang hi chipchiar takin sawi ila!

I taksaa thisen siamna hmunpui ber chu ruhro (bone marrow) a ni. Factory ang mai a ni. He factory hian thisen sen, thisen var, leh platelet ang chi thil a siam a, chu chuan kan taksa a ti na thei a ni. He factory hian hna a thawk tawh lo thut a, a nih loh leh a thawk tha lo a nih chuan eng nge thleng ang tih han ngaihtuah teh? Chu chu bone marrow failure chu a ni. Hemi chungchang hi chipchiar deuhin han sawi ila .

Bone Marrow Failure hi eng nge ni?

A awlsam zawngin, bone marrow failure chu i bone marrow-in taksa tana thisen sen hrisel, thisen var, platelet a siam chhuah theih loh vang a ni. Heng cell chi hrang hrangte hian kan taksaah hian chanvo bik an nei vek a.

• Thisen sen (red blood cells) hi taksa pum puia oxygen phurtu a ni. An hniam chuan hahna leh paleness i nei thei.
• Thisen var (white blood cells) hi kan taksaa sipai awm ang mai a ni. Infection an do thin. An tlem chuan damlohna chance a sang hle.
• Platelets hian thisen zam a pui a, chu chu i hliam chuan thisen a chhuak tawh lo tihna a ni. I platelet count a tlahniam chuan hliam te tak te pawhin thisen a chhuah tam thei hle.

Bone marrow failure hi damdawi lam thil dang avanga harsatna a ni fo thin. Mahse a châng chuan a chhan hriat lohvin a thleng thei a ni. Doctor-te chuan damdawi leh enkawlna chi hrang hrang hmangin natna lan chhuah dan an enkawl thei a ni. Mahse, he natna hi hun rei tak chhunga enkawlna awmchhun chu allogeneic stem cell transplant a ni .

Hetiang dinhmun hi eng chi hrang hrang nge ni ber?

Bone marrow failure hi chi hnih a awm a, chungte chu:

1. Acquired: Mithiamte chuan he natna hi eng nge a thlen tih chiang takin an hre lo. Mahse zirchianna atanga a lan dan chuan natna thenkhat emaw, chemical thenkhat nena inzawmna emaw, damdawi thenkhat hman avanga lo awm thei a ni. Hetiang chi hi hun kal zelah a lo thang zauh zauh thin .

2. Inherited: Hei hi i nu leh pa pakhat emaw, pahnih emaw hnen atanga genetic changes (mutations) i rochun chuan a thleng thin. Doctor te chuan hei hi Bone Marrow Failure Syndrome an ti a .

Hetiang dinhmun hi engtiang chiahin nge a awm?

Hei hi a takah chuan thil awm dan tlangpui a ni lo. Entîr nân, kum tin United States-a nau piang maktaduai zînga 65 vêl chuan hereditary bone marrow failure syndrome an nei a ni. Entirna dang chu thlahtute atanga lo piang, ``Fanconi Anemia'' tih hi a ni.Million zinga panga zinga pakhat velin a vei a ni. Chuvangin i hmu thei, hei hi a tlem deuh.

Eng nge a symptoms chu ni thei ang?

Bone marrow failure chhan a zirin symptoms a inang lo thei. Entirnan, hereditary bone marrow failure nei mi pakhat chuan kum hnih a tlin atang tawhin a natna lan chhuahna a nei tan thei a ni. Mahse, zirchianna atanga a lan dan chuan he natna hi acquired type nei mi pakhatin kum 20 leh 25 inkar atang tawhin a lan tan tir thei a, kum 65 atang pawhin a lan tan thei a, engtik atanga tan pawh ni se, a lan chhuah tam ber chu:

• Ruh a na
• Thisen chhuak tam lutuk (e.g. hnar thisen chhuak, hmui thisen chhuak, thlasik laiin thisen chhuak nasa) .
• Chauhna leh hah lutuk
• Khawsik fo thin
• Bacteria natna kai fo thin
• Lu na
• Thâwk harsa (Dyspnea) .
• Vun rawng chu a hma aia a sen deuh deuh
• Vun hnuaia hmun sen te te (Petechiae) .
• A chhan hriat loh taksaah hliam tuar

Heng symptoms pakhat emaw a aia tam emaw i neih chhunzawm chuan doctor pan ngei ngei tur a ni.

Engvangin nge he ruhro hi a hlawhchham? A chhante chu engte nge ni?

A hnuaia thilte hian ruhro (bone marrow failure) i neih theihna tur a tipung thei a ni:

• Bone Marrow Failure Syndrome (chu chu rochun atanga lo piang) i nei a.
• Thisen cancer emaw, thisen natna dang emaw, thisen natna thenkhat, cancer emaw, infection emaw i nei a (hengte hi a hnu lama lo awm, chu chu `(Acquired)` type-a mi an ni tihna a ni).
• Chemical leh damdawi thenkhat nena inzawmna (hei pawh hi `(Acquired)` category-ah a tel).

A châng chuan, he dinhmun hi chhan hriat lohvin a awm thei bawk. Doctor-te chuan " idiopathic bone marrow failure" an ti bawk. Researcher te chuan autoimmunity leh bone marrow failure hi inzawmna a awm niin an ngai. Autoimmunity chu i taksa ngeia immune system-in i ruhro a rawn beih palh hi a ni.

Inherited Bone Marrow Failure Syndrome atanga lo chhuak a ni

Hengte hi rochun, a inzawm, a nih loh leh a lan chhuah dan inang lo thenkhat chu an ni:

• Congenital Agranulocytosis (Kostmann Syndrome): Hei hian thisen var chi khat neutrophils an tih chu a nghawng a.
• Congenital Amegakaryocytic Thrombocytopenia (CAMT): Hetiang dinhmunah hian i ruhroah hian platelet siamna atana puitu megakaryocytes an tih cells a tlahniam a.
• Diamond-Blackfan Anemia (Diamond-Blackfan Anemia): Hei hi natna khirh tak a ni a, thisen sen (red blood cells) siamchhuahna a tichhe thei a ni.
• Dyskeratosis Congenita (Dyskeratosis Congenita - `(Dyskeratosis Congenita)`) tih hi a ni a, a natna hi a lar hle.Hetiang natna lan chhuah danah hian vun inthlak danglamna a tel a, hei hi ruhro (bone marrow failure) chhinchhiahna hmasa ber a ni thei.
• Fanconi Anemia (Fanconi Anemia): Hei hi bone marrow failure syndrome awm tam ber a ni.
• Reticular Dysgenesis (Reticular Dysgenesis): Hei hi Severe Combined Immunodeficiency (SCID) chi khat a ni a, a natna hi a chi khat a ni. Hetiang dinhmunah hian i taksa hian T-cell puitling a siam tling lo.
• Severe Congenital Neutropenia (Severe Congenital Neutropenia): Hetiang dinhmunah hian i neutrophils, thisen var chi khat chu a pangngai aia hniam a ni.
• Shwachman-Diamond Syndrome (Shwachman-Diamond Syndrome): He natna hian i pancreas, ruhro leh ruhte a nghawng a ni.

I nu leh pa zinga pakhatin he natna leh a lan chhuah dan (hei hi ``Autosomal Dominant'' an ti a) a neih chuan, he natna hi i vei theihna chance leh bone marrow failure syndrome i neih theihna chance 50% i nei a ni.

Mahse, i nu leh pa pahnih hian bone marrow failure syndrome chi khat nena inzawm gene mutation an keng tel a, mahse an pahnih hian symptom an nei lo a nih chuan (hei hi ``Autosomal Recessive'' an ti a), chu natna vei theihna chu 25%-ah a tlahniam a ni.

Thisen Cancer awm thei te

A hnuaia cancer chi hrang hrang zinga pakhat i neih chuan bone marrow failure i nei sang zawk a ni:

• Lymphoma natna a ni
• Multiple Myeloma natna a awm
• Myelodysplastic Syndrome natna a ni

Thisen lama harsatna dang

Mi thenkhat chu pianpuiin heng natna nei an ni. Mi dangte chuan hun kal zelah an siam chhuak thin. Chung zingah chuan:

• Aplastic Anemia (Aplastic Anemia): Hei hi thisen lama natna hmuh tur awm lo tak a ni. I ruhro a tichhia a, thisen cell leh platelet thar a siam loh nan a veng bawk. Doctor-te chuan ruhro (bone marrow failure) chungchang an sawi hian "aplastic anemia" tih hi an hmang mai thei.
• Cytopenias (Cytopenias): Hei hian autoimmune neutropenia, idiopathic neutropenia, leh granular lymphocyte leukemia lian tak tak te a huam a ni.
• Paroxysmal Nocturnal Hemoglobinuria (Paroxysmal Nocturnal Hemoglobinuria): Hetiang dinhmunah hian i immune system hian thisen sen (red blood cells) a tichhia a ni.
• Pure Red Cell Aplasia (Pure Red Cell Aplasia): Hei hi inherited condition a ni bawk a, thisen sen siam chhuahna a tichhe thei a ni.

Viral Infection awm thei te

A hnuaia virus infection te hian i risk a tipung thei bawk:

• Cytomegalovirus `(Cytomegalovirus)` hmanga natna hrik pai a ni
• Epstein-Barr Virus atanga lo chhuak a ni
• Mihring taksa chak lohna hrik (HIV) .
• Parvovirus B19 `(Parvovirus B19)` tih hi a ni
• Viral Hepatitis natna a ni

Cancer natna atana chemotherapy leh/ emaw radiation therapy hmanga enkawlna hian ruhro (bone marrow failure) i neih theihna a tipung thei bawk. Tin, insecticide leh pesticide thenkhata hman chemical leh solvent te nena inzawmna i neih hian i hlauhawmna a tipung thei bawk.

Hetiang dinhmun hian eng harsatna nge a thlen?

Hei hi natna khirh tak a ni a, nunna atana hlauhawm thei a ni . Enkawlna hnuah pawh a hnuaia harsatnate hi a awm thei a ni:

• Bacteria emaw virus emaw atanga kai theih
• Thisen chhuak
• Thisen cancer, entirnan, acute myeloid leukemia emaw myelodysplasia emaw
• Cancer natna dang, entirnan osteosarcoma leh rhabdomyosarcoma te
• Squamous Cell Carcinoma natna a ni

Engtin nge hei hi i diagnose?

Doctor chuan i symptoms te, i medical history te, leh i chhungte medical history te a zawt hmasa ang che. Physics exam pawh an ti dawn a ni. Thisen test leh imaging test pawh an order thei bawk.

Thisen Test neih a ni

Thisen test-ah hian:

• Ceruloplasmin Test hmanga enfiah a ni
• Thisen chhiar kim (CBC) 1.1.
• Ferritin Test a ni
• Folate tlakchhamna Test
• Vitamin B12 tlakchhamna test

Imaging Test te pawh a awm

Imaging test-ah hian:

• Magnetic Resonance Imaging (MRI - `(Magnetic Resonance Imaging)` scan) hmanga tihchhuah a ni.
• Positron Emission Tomography (PET - `(Positron Emission Tomography)` scan) a ni a, a chhuahna tur hmun leh a hmanna tur hmun a awm lo.
• Ultrasound hmanga enfiah `(Ultrasound)` tih a ni

Thisen test leh imaging test result a\angin i doctor chuan bone marrow biopsy neih a rawt thei ang . Bone marrow failure thlentu genetic mutations hriat theihna turin genetic test neih a rawt thei bawk.

I doctor chuan natna thenkhat hriat theihna tur emaw, natna dang a awm loh nan emaw test a ti thei bawk. Test tin an tih chhan leh test-in eng nge a lantir theih tih an hrilhfiah ang che.

Enkawlnate chu engte nge ni?

I enkawlna tur chu thil engemaw zatah a innghat ang a, chung zingah chuan:

• Bone marrow failure chi hrang hrang (i.e. inherited emaw acquired emaw pawh nise).
• I dinhmun nasat dan.
• I kum leh i hriselna zawng zawng.
• I symptoms te chu.

Enkawlna kawng hrang hrangte chu:

• Antibiotics , antifungals, leh natna hrik dona atana antiviral damdawi te.
• Thisen pek hian red blood cell count a tipung a, thisen chhuah leh hah ang chi symptoms a tihziaawm bawk.
• Bone marrow stimulants hian i bone marrow chu thisen cell tam zawk siam turin a pui thin.
• Immunosuppressants hian i immune system in i ruhro a beih loh nan a pui thin.
• Allogeneic Stem Cell (Bone Marrow) hmanga siam chhuah .

Enkawlna hian side effect emaw complication emaw a nei em? (Enkawlna lama harsatna awm thei) .

Enkawl dan azirin harsatna leh side effect a inang lo a, mahse stem cell (bone marrow) transplant hi harsatna lian tham a thlen tam ber a ni. Chung zingah chuan graft-versus-host disease (GVHD) leh infection te pawh a tel.

Hetiang dinhmun nen hian nun chu eng ang nge ni ang? (Prognosis) tih a ni.

Enkawlna hnua i beisei theih tur (prognosis) chu thil engemaw zatah a innghat a, chung zingah chuan natna chi hrang hrang, i kum leh hriselna zawng zawng, leh i taksain enkawlna a dawn dan te a innghat a ni.

A tlangpuiin, ruhro (bone marrow failure) nei te chuan damdawi lam enkawlna leh puihna kalpui zel an mamawh a ni . Mahse mi zawng zawng hi kan inang vek lo. I dinhmun chiang takin hria la, i enkawl hmasak ber hnuah eng nge thleng leh ang tih doctor zawt rawh.

Bone marrow failure hi thihna thlentu a ni em? (Terminal a ni em?)

Hei hi natna khirh tak, nunna atana hlauhawm tak a ni . Bone marrow failure nei tam zawk hi specialist tawnhriat ngah tak tak oncologist leh hematologist te enkawl an ni.

Heng damlote dam rei zawng hi eng nge sawi theih ang? (Nun rei zawng) .

Dam rei zawng chu mi pakhatin natna engemaw enkawl a nih hnua eng chen nge a dam dawn tih chhut a ni. Bone marrow failure nei te dam rei zawng hi thla engemaw zat atanga dam chhung zawng a ni thei.

Mahse, i mi bik a, i thil tawn chu midang zawng zawng aiin a danglam thei bawk. I doctor hnenah eng nge i beisei theih ang tih zawt la, eng chen nge i dam theih beisei ang tih zawt rawh. Anni hi i thu hriatna tha ber an ni a, a chhan chu nangmah leh i dinhmun an hriat vang a ni.

Risk hi tihziaawm theih a ni em? (Risk tihziaawmna) .

Vanduaithlak takin, inherited bone marrow failure hlauhawmna tihziaawmna tur a awm lo. Mahse, bone marrow function ti tlem thei nia hriat chemicals te pumpelh hian acquired bone marrow failure thenkhat a awm theihna a ti tlem thei a ni. Tin, enkawl hmasak hian i natna lan chhuah dan a tiziaawm thei a, i nunphung pawh a ti tha thei bawk.

Engtin nge ka in enkawl ang? (Mahni Inenkawlna) .

A tha ber chu i hriselna zawng zawng enkawl hi a ni . Entirnan, zuk leh hmuam hmang suh, zu in khap emaw, bansan emaw. Thurawn dang thenkhat chu hetiang hi a ni:

• Doctor hmu fo ang che. Bone marrow failure hi dam chhung zawnga natna a ni thei. Enkawlna kalpui zel i mamawh mai thei. Hriselna lama harsatna thar a thlen thei bawk. Chuvangin checkup neih fo a pawimawh. I doctor chuan i hriselna zawng zawng a enfiah ang a, harsatna thar i neih chuan a enkawl ang.
• Ei leh in hrisel ei rawh. I enkawlna atanga i symptoms leh side effects te hian ei tur a ti harsa thei a, i ei duhna a hloh thei bawk. I mamawh tur nutrition i dawng em tih i chiang lo a nih chuan nutritionist nen inbia ang che. Anni chuan rawtna dik tak an pe thei che a ni.
• Exercise engemaw zat nei rawh. Bone marrow failure ang chi hriselna lama harsatna lian tak neih hi a buaithlak thei hle. Exercise hi stress tihziaawmna tha tak a ni. Exercise thar i tan hmain i doctor nen inbiakna neih hmasak phawt ang che.

Engtikah nge Emergency Treatment Unit (ETU) ah i kal a ngaih ?

Bone marrow failure i neih chuan natna hrik kai leh thisen chhuahna lama harsatna i neih theihna a sang zawk. A hnuaia thilte hi i tawn chuan emergency room-ah i kal tur a ni:

• Painkiller hman tlanglawn tak ei hnuah pawh khawsik a tlahniam lo
• Khua a vawt leh khur (Chills) .
• Thisen chhuah tihtawp theih loh

Doctor hnenah eng zawhna nge ka zawh ang?

Natna rochun thenkhat avangin bone marrow failure i nei thei bawk. Mahse, a châng chuan chhan hriat lohvin a thleng thei a ni. I doctor hnenah hetiang zawhna hi i zawt thei ang:

• Hetiang bone marrow failure hi ka neih chhan i hria em?
• Eng enkawlna kawng nge ka neih?
• Chatuan atan enkawlna ka lak a ngai dawn em ni?
• Hei hi tihdam theih a ni em?

Bone marrow failure i neih chuan hriselna lama harsatna lian tak, rochun natna, thisen lama harsatna, a nih loh leh cancer ang chi i tawk tawh mai thei. He diagnosis thar hian zawhna leh ngaihtuahna thar tam tak a siam thei che a, chu chu a buaithlak thei hle. Hei hi nunna atana hlauhawm tak a nih theih avangin lungkhamna neih hi thil pangngai a ni. I medical team nen eng nge i beisei tur leh eng nge i tih leh tur tih sawipui rawh. I zawhnate chu lawm takin an chhang ang.

Thuhmahruai angin hre reng ang che (Take-Home Message) .

Bone marrow failure hi natna khirh tak leh buaithlak tak a ni . Mahse, a chhan awm thei tam tak a awm tih hre reng la, enkawlna leh enkawl dan hrang hrang a awm bawk. A pawimawh ber chu a hmaa diagnosis neih a, specialist dik tak hnen atanga enkawlna zawng a, chu thupekte chu zawm chhunzawm zel hi a ni.Hemi chungchangah hian zawhna emaw, ngaihtuahna emaw i neih chuan i doctor nen inbiakna neih hlau suh. I tanpui turin an awm reng a ni.

` Bone Marrow Failure, Red Blood Cells, White Blood Cells, Platelets, Stem Cell Transplant, Bone Marrow Natna te