Myelofibrosis: He thisen cancer chungchang hi kan hre tak tak em?

Chhan awm lovin i chau leh chau i ti fo em? I taksa pum puiah hliam i nei em? I pum veilam sir ah hian a rit deuh emaw, a khat ang tih emaw i nei em? Heng thilte hnung lamah hian kan ngaihtuah ngai loh chhan lian tak a awm mai thei. Chu chu myelofibrosis an tih natna a ni. He hming i hriat hian hlau suh. Eng nge a nih, engvangin nge a lo awm tih, a natna lan chhuah dan te, a enkawl dante chu i hriatthiam theih tur angin kan sawi dawn a ni.

A awlsam zawngin, myelofibrosis chu eng nge ni?

Kan taksa chhunga ruh lian tak takte hi thisen siamna factory angin ngaihtuah teh. Heng factory te hi bone marrow kan ti thin . He ruhro chhungah hian kan taksa tana pawimawh thisen cell chi thum zawng zawng hi siam chhuah vek a ni.

1. Thisen sen (Red Blood Cells): Taksa pumpuiah oxygen a keng tel a.

2. White Blood Cells: Natna leh hrik te a do thin.

3. Platelets: Thisen zam a siam a, thisen chhuah a titawp.

Myelofibrosis hi thisen cancer chi khat hmuh tur awm lo tak a ni a, thisen siamtu factory ruhro chu zawi zawiin scar tissue-in a khat ta a ni. Weeds-in field tha tak a luah zauh zauh ang bawkin, he scar tissue hian bone marrow-in thisen cell a siam dan pangngaiah a veng a ni.

He hna hi a thawh that loh avangin kan spleen , chu chu pum veilam sir chunglam organ a ni a, he hna hi puih a tum a ni. Chu chu spleen hian thisen a siam tan tihna a ni. Mahse chu chu a hna ber a ni lo. Chumi avang chuan spleen chu a overload a, zawi zawiin a lian chho zel a, a lo hring tan bawk. Damlo tam tak chuan he spleen swelling hi symptoms an nei thin.

Hei hi dam chhung zawnga natna a ni tlangpui a, a chang chuan zawi zawiin a kal zel. Mahse, a chang chuan a zual thuai thei a, mi thenkhatah chuan acute leukemia thlengin a kal thei bawk. Chuvangin i doctor chuan i natna hmuhchhuah hnuah uluk takin a lo enkawl ang che.

Myelofibrosis chi hrang hrang lian ber ber a awm em?

Ni e, hei hi chi hnih lian tak a awm.

• Primary Myelofibrosis: Hei hi a tam ber a ni. Amah chauhin a lo awm a, natna dangin a nghawng lo.
• Secondary Myelofibrosis natna hi a awm a:Hei hi thisen kaihhnawih natna dangte a nasat chuan a thleng thin. Entirnan, Essential Thrombocythemia emaw Polycythemia Vera emaw vei chuan hun kal zelah myelofibrosis a nei thei a. Heng natna zawng zawng hi thisen cancer pawl pakhat Myeloproliferative Neoplasms (MPNs) an tih zinga mi an ni. A awlsam zawngin, heng natna zawng zawng hian ruhro hian thisen cell chi khat emaw, a aia tam emaw a siam chhuah tur aia tam a siam chhuak a ni.

He natna lan chhuah dan engte nge ni? Engtin nge kan hriat?

A tam zawkah chuan he natna hi a lo vei zauh zauh thin. Chuvangin, kum tam tak chhung chu natna lan chhuah lohvin a awm thei a ni. Symptoms a lo lang tan chuan ka rilruah a lo lang hmasa ber chu hah lutuk hi a ni . Hei hi taksaa thisen sen (red blood cells) a tlakchham vang a ni a, chu chu thisen tlakchhamna (anemia) a ni. Hemi rual hian, i rilru a lian lutuk avangin i pum veilam chunglam chu a rit, a na emaw, a khat emaw a awm thei bawk.

A hnuaia table-ah hian eng symptom dang nge awm tih i en ang u.

Engvangin nge hetiang hi a lo awm? A chhan engte nge ni?

Doctor-te chuan primary myelofibrosis awm chhan dik tak chu an la hre lo a, mahse a thlen dan erawh an hrethiam hle.

Chu chu ruhroa thisen siamtu cell hmasa ber stem cell-a gene pakhat mutation aṭangin a inṭan a ni . Gene dik lo nei cell chu cancer cell a lo ni ta a, rang takin a inthen darh a, a copy a siam ta a ni. Heng cancer cell te hian ruhro zawng zawng an luah khat vek a ni.

Doctor-te chuan he natna vei tam tak hian an gene-ah hian mutations common engemaw zat an nei tih an hmuchhuak a. Chung zinga pawimawh ber chu:

• JAK2 (JACK-TWO) chuan a rawn ti a.
• CALR (Cal-R) a ni.
• MPL

Heng genetic mutations te hi ruhro (bone marrow)-a he natna thlentu a ni.

Tute nge he natna vei theihna sang zawk?

He natna hi tu pawhin vei thei mah se, mi thenkhat chu an hlauhawmna a sang zawk.

• Kum 60 chunglam: He natna hi kum upa lam zingah hmuh chhuah a ni ber.
• Thisen natna dang awmna: 1.1.`Essential Thrombocytosis` emaw, a hmaa kan sawi tawh `Polycythemia Vera` ang chi natna nei tan.
• Ionizing radiation nena inzawmna: Radiation dose sang tak nena inzawmna. (Hei hi a tlem hle).
• Chemical thenkhat nena inzawmna: Hun rei tak chhunga factory-a hman tur chemical, benzene ang chi (hei pawh hi a tlem hle).

Natna avanga harsatna awm thei te chu engte nge ni?

Myelofibrosis a lo zual zel chuan harsatna hrang hrang a thleng thei a ni.

Thisen cell siam chhuah a tlahniam

Scar tissue chu bone marrow-ah a lo awm khawm chuan thisen cell hrisel a siam thei lo. Hei hian thisen sen a tlahniam thei a, thisen tlakchhamna a thlen thei a, platelet a tlahniam thei bawk a, thisen chhuah theihna a tipung thei bawk.

Bone marrow atanga extrahepatic blood cells siam chhuah

Bone marrow hian a hna a thawh theih loh chuan spleen leh liver ang chi organ te hian thisen an siam tan thin. Hei hian chu taksa pengte chu a ti hring thei a, an hnathawh a tichhe thei bawk.

Portal Vein-a Thisen sang a awm

Portal vein hi thisen kalna kawng lian ber a ni a, chu chuan thisen chu spleen atanga liver-ah a phur a ni. Spleen lian zawk chuan he vein kal tlanga thisen kal chu a tikhawlo thei a, a chhunga pressure a tisang thei a ni. Hei hian thisen chhuahna nasa tak a thlen thei a ni.

Acute leukemia ah a chang ta

Damlo thenkhatah chuan myelofibrosis hi a tawpah chuan leukemia chi khat na tak leh darh chak takah a lo chang thei a, chu chu Acute Myeloid Leukemia (AML) tih a ni.

Engtin nge doctor hian he natna hi dik taka a hriat theih ang ?

I natna lan chhuah dan a ngaihthlak hnuah leh a enfiah hnuah doctor chuan i natna hriat chian nan test eng emaw zat a ti ang.

1. Thisen Test: Hengte hian i thisen cell count (red, white, platelet) a normal em tih leh a pangngai lo tih a enfiah thin. Tin, thisen cell pianzia chu a dik lo em tih an enfiah bawk.

2. Imaging Test: CT scan emaw ultrasound scan emaw hmangin i spleen emaw liver emaw a lian em tih hriat theih a ni. MRI scan hian ruhroah hliam a awm leh awm loh a hriat theih bawk.

3. Bone Marrow Biopsy: Hei hi natna finfiah nan test pawimawh ber a ni. Hetah hian ruhro sample tlemte, a tlangpuiin hip ruh emaw ruh lian dang emaw atanga lak a ni a, light anesthetic hnuaiah lak a ni. Lab-ah thawn a ni a, scar tissue engzat nge awm tih chiang takin a hriat theih nan, cancer cells a awm em tih te, a hmaa kan sawi tawh `JAK2` ang chi genetic mutations a awm em tih te hriat theih a ni.

Heng test result a\ang hian i doctor chuan i natna chu a bul tanna (prefibrotic) emaw, a zual (overt) emaw a nih leh nih loh a hre thei ang. Tin, i natna lan chhuah dan leh genetic mutation chi hrang hrang a zirin i risk level (low, intermediate, emaw high-risk) an zirchiang bawk ang. Hetiang risk level atanga enkawl tura ruahman a ni.

Hei hi eng enkawlna nge ni?

Enkawl dan tur chu i natna hlauhawmna level, i natna lan chhuah dan nasat dan, i kum leh i hriselna zawng zawngah a innghat a ni.

Intermediate leh high-risk condition te tan hian enkawlna hrang hrang a awm a. Enkawlna tum ber chu natna lan chhuah dan (symptoms) control a, natna a zual zel loh nan a ni.

Stem Cell thlak danglam a ni

Hei chauh hi myelofibrosis tidam thei tur enkawlna awmchhun a ni. Mahse, a risky hle a, side effect na tak a nei bawk. Chuvangin damlo zawng zawng tan a tha lo. A tlangpuiin doctor-te chuan he enkawlna hi risk sang tak, thalai leh natna khirh dang nei lo tan an rawt thin.

Hei hian damlo ruh natna nei chu tihchhiat vek a, damlo hnenah donor hrisel tak hnen atanga stem cells hmuh te pek a huam a ni.