I ruhro hian fibrous tissue a siam bawk em? Myelofibrosis chungchang hi i zir dawn teh ang!

Natna myelofibrosis an tih hi i hre tawh em? A nihna takah chuan thisen cancer chi khat hmuh tur awm lo tak a ni. A awlsam zawngin, i ruh nêm tak, spongy ang maia awm, bone marrow , chu fibrous scar tissue- in a thlak hunah a ni. Hun rei tak chhunga leukemia chi khat a ni bawk a, myeloproliferative disorders tih pawlah a tel bawk. Myeloproliferative disorders chu ruhro, thisen cell siamna hmuna thisen cell tam lutuk siam chhuah a ni.

Myelofibrosis hi eng nge ni tak tak?

Hei hi chipchiar zawkin han thlir ila. I ruhroah hian thisen siamtu stem cells a awm a . Heng stem cells te hi a hnuah thisen sen, thisen var, leh platelet ah te an lo thang lian a. Mahse myelofibrosis vei mi pakhatah chuan heng stem cell pakhata genetic material, a nih loh leh DNA- ah hian mutation a awm a. Hei hian cell chu cancer cell chhia a lo ni ta a ni. Chumi hnuah chuan he cell chhia hian a inthen darh a, chu mutation chu a siam cell thar zawng zawngah a pe chhuak ta a ni.

Hun a kal zel a, heng cancer cell pangngai lo te hi an pungkhawm nasa hle. Heng cell thenkhat hian ruhro (bone marrow) ah inflammation a siam thin. He inflammation hi a ni a, a hmaa ka sawi tawh scar tissue hi a lo piang ta a ni. Chuvangin, he scar tissue leh cancer cell tam lutuk avang hian ruhro hian thisen cell hrisel siam theihna a hloh ta a ni.

Myelofibrosis chi hrang hrangte chu engte nge ni?

Myelofibrosis hi chi hnih a awm a.

• Primary myelofibrosis: Hei hi a takin a lo awm a, natna dang nena inzawmna nei lo a ni.
• Secondary myelofibrosis: Hei hi thisen lama harsatna dang avanga lo awm a ni. Entirnan, natna chi hrang hrang, primary thrombocytosis emaw polycythemia vera emaw avanga lo awm thei a ni. He secondary type hi damlo hmuhchhuah zinga 10% leh 20% inkar a ni.

He natna Myelofibrosis an tih hi engtiang chiahin nge a hluar?

Myelofibrosis hi a takah chuan a awm lo hle . Entirnan, United States-ah chuan he natna hi kum khatah mi 100,000 zinga 1.5 velin a vei tih an sawi a. Kum eng pawh ni se, tu pawh a nghawng thei. Kum inthlauhna a awm lo a, mahse kum 50 chunglam zingah a awm tam zawk a, naupang tê têah myelofibrosis a lo awm chuan kum 3 an tlin hmain hmuhchhuah a ni tlangpui.

Myelofibrosis hian i taksaah eng nghawng nge a neih theih?

Hetianga thisen cell te hi a pangngai lo taka siam chhuah a nih chuan harsatna chi hrang hrang a awm thei a ni. Eng nge an nih hi han thlir ila:

• Anemia: Hei hi thisen zam (red blood cells) awm tawk lohna dinhmun a ni. I hriat angin thisen sen (red blood cells) te hi taksa pum puia oxygen phurtu an ni. Chuvangin red cells a tlem chuan taksa tissue te hian oxygen an hmu tlem hle. Hei hian hahna, chak lohna, leh thawk hah ang chi symptoms a thlen thei a ni .

• Thrombocytopenia: Hei hi platelet tlem lutuk awmna a ni. Platelets hi hliam a awm chuan thisen zam tura puitu a ni. Chuvângin, i platelet count a tlem chuan i taksaah thisen chhuak leh hliam a tam zâwk thei a ni .

• Splenomegaly: I spleen hian thisen cell count a control a, i taksa atanga thisen sen chhia chhia te a paih chhuak thin. Chuvangin thisen cell pangngai lo i siam tam lutuk chuan i spleen hian hna a thawk nasa zawk a ngai a ni. A lian thei hle. I rilru a lo lian chuan i pum veilam chunglam chu a khat emaw, a nuam lo emaw a ni thei .

• Extramedullary hematopoiesis: Hei hi ruhro pawn lama thisen siamtu cell , taksa hmun danga awmte lo piang chhuak a ni . Heng cell te hi lung, chaw kawng, ruhro, thluak emaw, lymph node emaw ang chi hmunah te an lo piang thei a ni. Heng cell te hi an inzawm khawm a, tumor an siam thei a, chu chuan taksa peng dang a rawn lut thei a, an hnathawh pawh a tibuai thei bawk.

• Portal hypertension: Hei hi i spleen atanga i liver-a thisen phurtu vein-a thisen sang tihpunna a ni. Hei hi a hmaa kan sawi tawh angin extramedullary hematopoiesis avanga veins tihchhiat vang a nih a rinawm ber.

Thil dang pakhat chu primary myelofibrosis vei zinga 12% vel tan chuan he natna hi thisen cancer chi khat na tak , acute myeloid leukemia -ah a lo chang thei a ni.

Eng thilin nge myelofibrosis hi a thlen?

Scientist te hian myelofibrosis hi eng nge a thlen tih chiang takin an la hre lo. Mahse gene thenkhat DNA inthlak danglamna nen a inzawm tih an hmuchhuak . Protein chi khat Janus-associated kinases (JAKs) an tih chu a inrawlh a ni. Heng JAKs protein te hian ruhro (bone marrow)-a thisen cell siamchhuahna chu a control a, cell-te hnenah signal a thawn a, chu chu inthen darh leh thanglian turin signal a thawn a ni. Heng JAKs protein te hi an overactive chuan thisen cell tam lutuk emaw, tlem lutuk emaw a siam thei a ni.

Myelofibrosis vei zinga 60% leh 65% inkar chuan JAK2 gene ah mutation an nei a . A dang 5% atanga 10%Myeloproliferative leukemia (MPL) gene-ah hian mutation a awm a. Tin, myelofibrosis vei zinga 20% leh 25% inkar ah Calreticulin (CALR) tih mutation hmuh tur a awm bawk.

Hemi atan hian tute nge hlauhawm ber?

A hnuaia thilte avang hian myelofibrosis vei theihna i nei sang thei a ni:

• Kum 50 aia upa i nih chuan .
• Thisen lama harsatna primary thrombocytosis emaw polycythemia vera emaw i neih chuan.
• Ionizing radiation emaw petrochemicals benzene leh toluene ang chi te i tawk tawh a nih chuan.

Myelofibrosis natna lan chhuah dan chu engte nge ni?

Myelofibrosis natna hi zawi zawiin a zual zel a , chuvangin kum tam tak chhung chu symptoms i nei lo mai thei. Damlo hmun thuma ṭhena hmun khat vel chuan a tir lamah chuan symptom an nei lo.

Symptoms a awm tak tak chuan a langsar ber chu hahna nasa tak (anemia vang) leh spleen lian lutuk hi a ni . Symptoms te chu hetiang hi a ni thei bawk:

• Hnathawh hahthlak tak a ni
• Khawsik
• Itching a ni
• Vun dum (pale skin) a ni
• Rihna tihtlem
• Zan a thawk hah
• Ruh emaw, ruh emaw a na
• Hri kai fo thin
• Spleen emaw liver emaw a lian zawk
• Sawifiah theih loh thisen zam
• Thisen chhuah emaw, hliam emaw danglam tak
• Pum leh chaw kawng (esophagus)-a thisen kalna (veins) lian tham (heng veins te hi a puak thei a, thisen a chhuak thei bawk).

He natna hi engtin nge kan hriat theih ang?

Doctor, a bik takin oncologist chuan taksa a enfiah ang che a, i damdawi lam chanchin a zawt ang che a, tuna i natna lan chhuah dan te a zawt ang che. Tin, spleen a lian leh thisen tlakchhamna chhinchhiahna a awm leh awm loh an enfiah bawk ang.

Tichuan, test hrang hrang an nei a, chu chuan natna dang a awm loh nan leh myelofibrosis i vei em tih finfiah a ni.

Thisen test a ni

• Complete blood count (CBC): Hei hian i thisena cell chi hrang hrang awm zat a teh a ni. I thisen sen (red blood cells) chu a pangngai aia hniam a nih chuan, emaw, i thisen var leh platelet te a dik lo a nih chuan myelofibrosis chhinchhiahna a ni thei.
• Peripheral blood smear (PBS): Hei hian i thisen cell lian leh te, a pianzia leh a mizia dangte chu a dik lo em tih a enfiah thin. Cell pangngai lo lang leh thisen puitling lo tam tak a awm chuan myelofibrosis chhinchhiahna a ni thei bawk.
• Thisen chemistry test dan: 1.1.Heng test te hian i taksa peng hrang hrang atanga thisena thil chi hrang hrang chhuak zat a teh thin. Hei hian organ pakhatin hna a thawh that zia a hriattir thei che a ni. Thisenah hian uric acid, bilirubin, leh lactic dehydrogenase ang chi thil tam lutuk hi myelofibrosis chhinchhiahna a ni thei bawk.

Bone marrow test neih a ni

• Bone marrow biopsy: Hei hi i ruhro sample tlemte lak a, microscope hmanga enfiah a ni. Cell te chu an zirchiang a, myelofibrosis i vei em tih an enfiah thin.
• Bone marrow aspiration: Hei hian ruhro atanga tui awm chu lakchhuah a, myelofibrosis chhinchhiahna a awm leh awm loh enfiah a ni.

Test dang neih belh a ngai mai thei

I natna hriat chian nan test dang neih belh a ngai mai thei:

• Gene mutation analysis: I doctor chuan i thisen leh ruhro cell te chu myelofibrosis nena inzawm gene mutations, JAK2, CALR, leh MPL te a test ang. Treatment thenkhatah chuan cancer cell JAK2 mutation nei te chu an target thin.
• Imaging procedures: I doctor chuan ultrasound hmangin spleen a lian em tih a enfiah thei ang. Tin, ruhro (bone marrow)-a scar tissue awm leh awm loh enfiah nan MRI an ti thei bawk. He scar tissue hi myelofibrosis chhinchhiahna a ni thei.

Myelofibrosis hi engtin nge enkawl a nih?

Symptoms i lan loh chuan enkawlna i mamawh lo mai thei. Amaherawhchu, enkawl vat ngai lo mah la, i doctor chuan i dinhmun chu a lo en reng ang.

Jakafi® (ruxolitinib), Inrebic® (fedratinib), leh Vonjo® (pacritinib) te hi U.S. Food and Drug Administration (FDA) -in a pawmpui damdawi a ni. Anni hi myelofibrosis hlauhawmna (moderate to high-risk myelofibrosis) enkawlna atan an hmang thin. Heng damdawi pathumte hian JAK inhibitor angin hna an thawk vek a ni . Chu chu, overactive Janus-associated kinase (JAK) signaling an tihziaawm tihna a ni. Chumi avang chuan heng damdawi te hian myelofibrosis nena inzawm symptoms te, spleen lian tak, zan lama thawk hah, riltam, taksa rihna tlahniam, leh khawsik te chu a tiziaawm thei a ni.

Mi tam tak tan chuan enkawlna tum ber chu myelofibrosis nena inzawm, thisen tlakchhamna leh spleen lian tham ang chite enkawl hi a ni.

Anemia enkawl dan

Anemia enkawlna tur a awm a, chungte chu:

• Androgens: Androgens danazol ang chi ei hian thisen sen (red blood cell) a tipung thei a ni.
• Immunomodulators: Heng damdawi te hian i immune system-in cancer cells a do theihna a tipung a, chu chuan symptoms a ti tlem a ni. Damdawi interferon, thalidomide (Thalomid®), leh lenalidomide (Revlimid®) te hi he class-ah hian an tel a ni. Prednisone ang chi glucocorticoids nen pawh pek theih a ni bawk.
• Chemotherapy damdawi: Chemotherapy damdawi thenkhat hian thisen cell count sang leh spleen lian vangin symptoms a tihziaawm thei. Hydroxyurea leh cladribine te hi chutiang damdawi chu an ni.
• Thisen pek: Thisen tlakchhamna nasa tak i neih chuan thisen pek fo hian i red blood cell count a tipung thei a ni.

Splenomegaly enkawl dan tur

JAK inhibitor, immunomodulators, leh chemotherapy damdawi te hi spleen lian zawk enkawl nan an hmang thin. A nasat chuan i spleen chu surgery hmanga lakchhuah (splenectomy) emaw, i spleen-a radiation therapy pek emaw a ngai mai thei.

Radiation therapy hi extramedullary hematopoiesis an tih, ruhro pawn lama thisen cell lo piang chhuak enkawlna atan pawh a ngai thei bawk.

Myelofibrosis hi a dam kim thei em?

He natna tihdamna tur hian allogeneic hematopoietic cell transplantation (HCT) chauh hi a ni mai thei. Mahse, hei hi risky procedure a nih avangin mi zawng zawng tan a tha lo.

Hetiang tih danah hian i thisen cell pangngai lo chu donor hrisel tak atanga cell te nen thlak a ni. Transplant hmain chemotherapy emaw radiation therapy emaw pek i ni ang a, chu chuan i cell natna chu tihhlum a ni ang. Tichuan, donor atanga cell thar lo chhuak chuan i taksa hnathawh chu a la thei ta a ni.

HCT enkawlna hian harsatna a thlen theihna a sang hle . Chuvangin mi thlan bikte tan chauh a inhmeh a ni. Damdawi lam harsatna dang nei tan chuan harsatna a thlen theihna a sang zawk. Hei hi i tlin leh tlin loh chu thil tam takah a innghat a, chu chu i kum, i natna lan chhuah nasat dan, leh enkawlna hlawhtlinna a awm theih dan te hi a ni.

He natna nena kan khawsak hian dam rei zawng hi eng ang nge ni?

Myelofibrosis hi cancer na tak a ni a . Heng damlote dam rei zawng hi kum ruk vel a ni tlangpui. Median survival rate tih hian mi thenkhat chu kum ruk aia rei lo an dam tihna a ni a, chutiang zat chu kum ruk aia rei an dam thung. Hei hi mi zawng zawng tan a inang lo, mi tin a inang lo.

I natna hmalam hun tur tichiangtu thil engemaw zat a awm a:

• i kum zat.
• I symptoms te chu.
• I thisen cell zat.
• Ruhro (bone marrow)-a hliam nasat dan.
• Genetic mutations a awm leh awm loh (JAK2, CALR, MPL).

A pawimawh ber chu i doctor nen i diagnosis hian i nun a nghawng dan tur chungchangah zalen taka inbiakna neih hi a ni.

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

Palliative care i hlawkpui theih leh theih loh doctor zawt rawh . Palliative care team-ah hian doctor, nurse, social worker leh specialist dangte pawh an tel thei. He natna nena nung tur hian i mamawh tur resources leh support an pe thei che a ni. I oncologist hnen atanga enkawlna i dawn bakah hian heng mite hi tanpuitu ropui tak an ni thei bawk. Palliative care specialist te hian cancer vei mi i nih angin i nunphung tihchangtlun nan an pui thei a ni.

A tawp berah chuan i hriat reng tur pawimawh ber berte chu

Myelofibrosis hi thisen cancer chi khat hmuh tur awm lo tak a ni a, i ruhro chu fibrous tissue in a thlak a ni. Hei hi natna khirh tak a ni a, damdawi lam enkawlna leh/ emaw enkawl chhunzawm zel a ngai a ni. I dinhmun azirin kum tam tak chhung chu symptom i nei lo mai thei. A dang erawh chuan symptoms te hi a lo thang chak hle a, nitin hna thawh a harsat phah thei bawk. Mahse, nitin nun tibuaitu symptoms control-na tur enkawlna a awm .

Chutih lai chuan, heng inthlâk danglamnate hmachhawn thei tûra ṭanpui theitu thilte chungchâng i doctor hnênah zawt rawh. Cancer natna vei nun i insiamrem theih nan palliative care leh support group te hi a \angkai hle.

Nangmah chauh i ni lo tih hre reng ang che. Doctor te, chhungte leh thiante te chu tanpui turin an awm vek a ni. Zawhna i neih chuan i doctor nen inbiakna neih hlau suh.

` Myelofibrosis, thisen cancer, ruhro, thisen tlakchham, spleen lian, JAK inhibitors, leukemia