Koj cov pob txha puas tsim cov nqaij fibrous? Cia peb kawm txog myelofibrosis!

Koj puas tau hnov ​​txog ib yam kab mob hu ua myelofibrosis? Qhov tseeb, nws yog ib hom mob qog ntshav uas tsis tshua muaj. Hais yooj yim xwb, nws yog thaum cov pob txha , uas yog qhov mos mos, thiab muaj cov pob txha me me, raug hloov los ntawm cov nqaij mos uas muaj cov hlab ntsha me me . Nws kuj yog ib hom mob leukemia ntev, thiab nws yog ib feem ntawm ib pawg hu ua myeloproliferative disorders. Myeloproliferative disorders yog thaum muaj ntau cov qe ntshav tsim tawm hauv cov pob txha, qhov chaw uas cov qe ntshav raug tsim tawm.

Myelofibrosis yog dab tsi tiag?

Cia peb saib qhov no kom meej ntxiv. Koj cov pob txha muaj cov hlwb uas tsim cov ntshav. Cov hlwb no tom qab ntawd loj hlob mus ua cov hlwb ntshav liab, cov hlwb ntshav dawb, thiab cov platelets. Tab sis rau ib tus neeg uas muaj myelofibrosis, kev hloov pauv tshwm sim hauv cov khoom siv caj ces, lossis DNA, hauv ib qho ntawm cov hlwb no. Qhov no ua rau lub hlwb ua rau lub hlwb mob qog noj ntshav tsis zoo. Lub hlwb tsis zoo no ces faib thiab xa cov kev hloov pauv mus rau txhua lub hlwb tshiab uas nws tsim.

Raws li lub sijhawm dhau mus, cov hlwb qog nqaij hlav no yuav loj hlob tuaj ua ntau heev. Ib txhia ntawm cov hlwb no ua rau mob o ntawm cov pob txha. Qhov mob o no yog qhov ua rau cov nqaij caws pliav uas kuv tau hais ua ntej tsim. Yog li, vim yog cov nqaij caws pliav no thiab cov hlwb qog nqaij hlav ntau dhau, cov hlwb pob txha poob nws lub peev xwm los tsim cov qe ntshav zoo.

Cov hom mob myelofibrosis tseem ceeb yog dab tsi?

Muaj ob hom mob myelofibrosis tseem ceeb.

• Mob myelofibrosis thawj zaug: Qhov no yog hom uas tshwm sim yam tsis muaj kev sib txuas nrog lwm yam kab mob.
• Kab mob myelofibrosis theem ob: Qhov no yog tshwm sim los ntawm lwm yam kab mob ntshav. Piv txwv li, nws tuaj yeem tshwm sim los ntawm cov kab mob xws li thawj tus kab mob thrombocytosis lossis polycythemia vera. Hom thib ob no suav txog li ntawm 10% thiab 20% ntawm cov neeg mob uas tau kuaj pom.

Kab mob no hu ua Myelofibrosis muaj ntau npaum li cas?

Kab mob Myelofibrosis yog ib yam mob uas tsis tshua muaj . Piv txwv li, hauv Tebchaws Meskas, muaj kev tshaj tawm tias muaj li ntawm 1.5 tus neeg ntawm 100,000 tus neeg mob txhua xyoo. Nws tuaj yeem cuam tshuam rau txhua tus neeg, tsis hais hnub nyoog li cas. Tsis muaj qhov sib txawv ntawm hnub nyoog, tab sis nws yuav raug kuaj pom ntau dua rau cov neeg hnub nyoog tshaj 50 xyoo. Yog tias myelofibrosis tshwm sim rau cov menyuam yaus, nws feem ntau raug kuaj pom ua ntej hnub nyoog 3 xyoos.

Myelofibrosis muaj peev xwm ua rau koj lub cev muaj teeb meem dab tsi?

Thaum cov qe ntshav tsim tawm tsis zoo li no, ntau yam teeb meem tuaj yeem tshwm sim. Cia peb saib seb lawv yog dab tsi:

• Kab mob ntshav tsis txaus: Qhov no yog ib qho mob uas tsis muaj cov qe ntshav liab txaus. Raws li koj paub, cov qe ntshav liab yog cov uas nqa oxygen thoob plaws lub cev. Yog li thaum tsis muaj cov qe ntshav liab txaus, cov nqaij ntawm lub cev tsis tau txais oxygen txaus. Qhov no tuaj yeem ua rau muaj cov tsos mob xws li qaug zog, tsis muaj zog, thiab ua tsis taus pa .

• Thrombocytopenia: Qhov no yog ib qho mob uas muaj cov platelets tsawg dhau. Cov platelets yog cov uas pab cov ntshav txhaws thaum muaj kev raug mob. Yog li thaum koj cov platelets tsawg, koj lub cev yuav yooj yim los ntshav thiab raug mob .

• Splenomegaly: Koj lub pores tswj cov qe ntshav thiab tshem tawm cov qe ntshav liab uas puas lawm ntawm koj lub cev. Yog li thaum koj tsim cov qe ntshav ntau dhau, koj lub pores yuav tsum ua haujlwm hnyav dua. Nws tuaj yeem loj hlob tuaj. Thaum koj lub pores loj hlob tuaj, koj yuav hnov ​​​​​​qhov puv lossis tsis xis nyob hauv sab laug sab saud ntawm koj lub plab .

• Extramedullary hematopoiesis: Qhov no yog kev loj hlob ntawm cov qe ntshav tsim sab nraum cov pob txha , hauv lwm qhov ntawm lub cev. Cov qe no tuaj yeem tsim nyob rau hauv qhov chaw xws li lub ntsws, txoj hnyuv plab, txoj hlab ntsha, lub hlwb, lossis cov qog ntshav. Cov qe no tuaj yeem sib sau ua ke los ua cov qog , uas tuaj yeem nkag mus rau lwm cov kabmob lossis cuam tshuam rau lawv txoj haujlwm.

• Mob ntshav siab hauv lub qhov (portal hypertension): Qhov no yog qhov ntshav siab ntxiv hauv cov leeg ntshav uas nqa cov ntshav los ntawm koj lub qhov ntswg mus rau koj lub siab. Qhov no feem ntau yog los ntawm kev puas tsuaj rau cov leeg ntshav vim yog cov ntshav tawm ntawm lub hlwb uas tau hais ua ntej.

Lwm yam yog tias rau li ntawm 12% ntawm cov neeg uas muaj tus kab mob myelofibrosis thawj zaug, tus mob no tuaj yeem hloov mus ua hom mob qog ntshav loj heev hu ua mob qog ntshav myeloid leukemia .

Dab tsi ua rau muaj mob myelofibrosis?

Cov kws tshawb fawb tseem tsis tau paub meej tias yog dab tsi ua rau muaj mob myelofibrosis. Tab sis lawv tau pom tias nws muaj feem cuam tshuam nrog kev hloov pauv hauv DNA ntawm qee cov noob caj noob ces . Muaj ib hom protein hu ua Janus-associated kinases (JAKs) koom nrog. Cov protein JAKs no tswj kev tsim cov qe ntshav hauv cov pob txha pob txha, xa cov cim mus rau cov qe ntshav kom faib thiab loj hlob. Yog tias cov protein JAKs no ua haujlwm ntau dhau, ntau dhau lossis tsawg dhau cov qe ntshav tuaj yeem tsim tau.

Nruab nrab ntawm 60% thiab 65% ntawm cov neeg uas muaj myelofibrosis muaj kev hloov pauv hauv JAK2 gene . Lwm 5% txog 10%Muaj kev hloov pauv hauv cov noob myeloproliferative leukemia (MPL). Tsis tas li ntawd, kev hloov pauv hu ua Calreticulin (CALR) pom muaj nyob nruab nrab ntawm 20% thiab 25% ntawm cov neeg mob myelofibrosis.

Leej twg muaj feem yuav raug qhov no tshaj?

Tej zaum koj yuav muaj feem yuav mob myelofibrosis ntau dua vim yog cov laj thawj hauv qab no:

• Yog tias koj muaj hnub nyoog tshaj 50 xyoo .
• Yog tias koj muaj teeb meem ntshav hu ua primary thrombocytosis lossis polycythemia vera.
• Yog tias koj tau raug rau cov hluav taws xob ionizing lossis cov tshuaj petrochemicals xws li benzene thiab toluene.

Cov tsos mob ntawm tus kab mob myelofibrosis yog dab tsi?

Myelofibrosis yog ib yam kab mob uas maj mam zuj zus tuaj , yog li ntawd tej zaum koj yuav tsis muaj tsos mob li cas rau ntau xyoo. Kwv yees li ib feem peb ntawm cov neeg mob tsis muaj tsos mob dab tsi thaum ntxov.

Yog tias muaj cov tsos mob tshwm sim, feem ntau yog qaug zog heev (vim yog ntshav tsis txaus) thiab lub qhov ntswg loj . Cov tsos mob kuj tseem yuav muaj xws li:

• Ua haujlwm nyuaj
• Ua npaws
• Khaus
• Daim tawv nqaij daj ntseg
• Kev poob phaus
• Kev hws hmo ntuj
• Mob pob txha los yog mob pob qij txha
• Kev kis kab mob ntau zaus
• Lub siab lossis lub raum loj dua
• Cov ntshav txhaws uas tsis paub meej
• Kev los ntshav lossis mob nqaij txawv txawv
• Kev loj hlob ntawm cov leeg ntshav hauv lub plab thiab txoj hlab pas (cov leeg ntshav no tuaj yeem tawg thiab los ntshav).

Yuav kuaj mob li cas?

Tus kws kho mob, tshwj xeeb yog tus kws kho mob qog nqaij hlav, yuav kuaj koj lub cev, nug txog koj keeb kwm kev mob nkeeg, thiab nug txog koj cov tsos mob tam sim no. Lawv kuj tseem yuav kuaj seb puas muaj tus po loj thiab cov cim qhia tias muaj ntshav tsis txaus.

Tom qab ntawd, ntau yam kev kuaj mob raug ua los txiav txim siab lwm yam mob thiab paub meej tias koj puas muaj myelofibrosis.

Kev kuaj ntshav

• Kev suav cov ntshav tag nrho (CBC): Qhov no ntsuas tus naj npawb ntawm ntau hom qe ntshav sib txawv hauv koj cov ntshav. Yog tias koj cov qe ntshav liab qis dua li qub, lossis koj cov qe ntshav dawb thiab cov platelets tsis zoo li qub, nws yuav yog ib qho cim ntawm myelofibrosis.
• Kev kuaj ntshav ntawm cov hlab ntsha (PBS): Qhov no yuav kuaj xyuas qhov loj, cov duab, thiab lwm yam ntxwv ntawm koj cov qe ntshav kom paub txog qhov tsis zoo. Yog tias muaj cov qe ntshav uas zoo li tsis zoo thiab muaj ntau cov qe ntshav uas tsis tau loj hlob, nws kuj tseem yuav yog ib qho cim ntawm myelofibrosis.
• Kev ntsuam xyuas cov tshuaj lom neeg hauv cov ntshav:Cov kev kuaj no ntsuas cov theem ntawm ntau yam tshuaj uas tso tawm rau hauv cov ntshav los ntawm koj cov kab mob. Qhov no tuaj yeem qhia koj tias lub cev ua haujlwm zoo npaum li cas. Cov theem siab ntawm cov khoom xws li uric acid, bilirubin, thiab lactic dehydrogenase hauv cov ntshav kuj tuaj yeem yog ib qho cim ntawm myelofibrosis.

Kev kuaj mob pob txha

• Kev kuaj cov pob txha hauv lub cev: Qhov no suav nrog kev coj me me ntawm koj cov pob txha hauv lub tshuab kuaj mob. Cov hlwb raug soj ntsuam kom paub seb koj puas muaj mob myelofibrosis.
• Kev rho cov pob txha tawm: Qhov no suav nrog kev tshem tawm cov kua dej tawm ntawm cov pob txha thiab kuaj nws rau cov cim ntawm myelofibrosis.

Tej zaum yuav xav tau kev ntsuam xyuas ntxiv

Tej zaum yuav xav tau kev kuaj ntxiv kom paub tseeb tias koj muaj mob:

• Kev tshuaj xyuas kev hloov pauv ntawm cov noob caj noob ces: Koj tus kws kho mob yuav kuaj koj cov ntshav thiab cov hlwb pob txha rau kev hloov pauv ntawm cov noob caj noob ces uas cuam tshuam nrog myelofibrosis, xws li JAK2, CALR, thiab MPL. Qee qhov kev kho mob tsom mus rau cov hlwb qog noj ntshav uas muaj kev hloov pauv JAK2.
• Cov txheej txheem thaij duab: Koj tus kws kho mob yuav ua ultrasound los xyuas seb puas muaj tus po loj. Lawv kuj tseem yuav ua MRI los xyuas seb puas muaj cov nqaij caws pliav hauv cov pob txha. Cov nqaij caws pliav no tuaj yeem yog ib qho cim ntawm myelofibrosis.

Yuav kho tus mob myelofibrosis li cas?

Yog koj tsis muaj tsos mob, tej zaum koj yuav tsis tas yuav kho mob. Txawm li cas los xij, txawm tias koj tsis xav tau kev kho mob tam sim ntawd los xij, koj tus kws kho mob yuav txuas ntxiv saib xyuas koj tus mob.

Jakafi® (ruxolitinib), Inrebic® (fedratinib), thiab Vonjo® (pacritinib) yog cov tshuaj uas tau txais kev pom zoo los ntawm Teb Chaws Asmeskas Lub Chaw Saib Xyuas Khoom Noj thiab Tshuaj (FDA) . Lawv siv los kho cov kab mob myelofibrosis uas muaj kev pheej hmoo nruab nrab mus rau siab. Tag nrho peb yam tshuaj no ua haujlwm ua JAK inhibitors . Ntawd yog, lawv txo qhov kev ua haujlwm ntawm Janus-associated kinase (JAK) signaling. Yog li ntawd, cov tshuaj no pab txo cov tsos mob uas cuam tshuam nrog myelofibrosis, xws li lub qhov ncauj loj, hws hmo ntuj, khaus, poob phaus, thiab kub taub hau.

Rau ntau tus neeg, lub hom phiaj tseem ceeb ntawm kev kho mob yog los tswj cov mob uas cuam tshuam nrog myelofibrosis, xws li ntshav tsis txaus thiab tus po loj.

Kev Kho Mob Rau Kab Mob Ntshav Tsis Txaus

Muaj cov kev kho mob rau anemia xws li:

• Androgens: Kev noj cov androgens xws li danazol tuaj yeem ua rau cov qe ntshav liab ntau ntxiv.
• Cov Tshuaj Kho Mob Tiv Thaiv Kab Mob: Cov tshuaj no ua rau koj lub cev tiv thaiv kab mob muaj peev xwm tawm tsam cov hlwb qog noj ntshav, yog li ntawd txo cov tsos mob. Cov tshuaj xws li interferon, thalidomide (Thalomid®), thiab lenalidomide (Revlimid®) yog cov tshuaj hauv pawg no. Lawv kuj tseem yuav muab ua ke nrog glucocorticoids xws li prednisone.
• Cov tshuaj kho mob qog noj ntshav: Qee cov tshuaj kho mob qog noj ntshav tuaj yeem txo cov tsos mob uas tshwm sim los ntawm kev nce ntxiv ntawm cov qe ntshav thiab lub qhov ntswg loj. Hydroxyurea thiab cladribine yog cov tshuaj zoo li no.
• Kev hloov ntshav: Yog tias koj muaj mob ntshav tsis txaus, kev hloov ntshav tsis tu ncua tuaj yeem ua rau koj cov qe ntshav liab ntau ntxiv.

Kev Kho Mob Splenomegaly

Cov tshuaj JAK inhibitors, cov tshuaj immunomodulators, thiab cov tshuaj chemotherapy yog siv los tswj tus po loj. Hauv cov mob hnyav, koj yuav tsum tau phais koj tus po tawm (splenectomy) lossis siv tshuaj tua kab mob rau tus po.

Kev kho mob hluav taws xob kuj tseem yuav tsum tau kho tus mob hu ua extramedullary hematopoiesis, uas cov qe ntshav tsim tawm sab nraud ntawm cov pob txha.

Puas kho tau myelofibrosis kom zoo tag nrho?

Kev hloov hlwb ntshav (HCT) tej zaum yuav yog tib txoj kev xaiv los kho tus kab mob no. Txawm li cas los xij, qhov no yog ib qho kev phais mob uas muaj kev pheej hmoo thiab tsis haum rau txhua tus.

Hauv txoj kev kho mob no, koj cov qe ntshav tsis zoo yuav raug hloov nrog cov qe ntshav los ntawm tus neeg pub dawb uas noj qab nyob zoo. Ua ntej hloov ntshav, koj yuav tau txais kev kho mob chemotherapy lossis kev kho mob hluav taws xob kom tua koj cov qe ntshav uas muaj mob. Tom qab ntawd, cov qe ntshav tshiab los ntawm tus neeg pub dawb tuaj yeem ua haujlwm ntawm koj lub cev.

Kev kho mob HCT muaj kev pheej hmoo siab ntawm cov teeb meem . Yog li ntawd, nws tsuas yog haum rau ib pawg neeg xaiv xwb. Kev pheej hmoo ntawm cov teeb meem tseem siab dua rau cov neeg uas muaj lwm yam mob. Seb koj puas haum rau qhov no nyob ntawm ntau yam, xws li koj lub hnub nyoog, qhov hnyav ntawm koj cov tsos mob, thiab qhov yuav ua tiav ntawm kev kho mob.

Lub neej ntev npaum li cas thaum muaj tus kab mob no?

Myelofibrosis yog ib yam mob qog noj ntshav loj heev . Feem ntau cov neeg mob no muaj sia nyob li ntawm rau xyoo. Feem ntau cov neeg muaj sia nyob nruab nrab txhais tau tias qee tus neeg muaj sia nyob tsawg dua rau xyoo, thaum cov neeg zoo sib xws muaj sia nyob ntau dua rau xyoo. Qhov no tsis zoo ib yam rau txhua tus, nws txawv ntawm ib tus neeg mus rau ib tus neeg.

Muaj ntau yam uas txiav txim siab qhov kev kwv yees ntawm koj tus kab mob:

• koj lub hnub nyoog.
• Koj cov tsos mob.
• Koj cov qe ntshav suav.
• Qhov hnyav ntawm cov qhov txhab hauv cov pob txha.
• Txawm muaj kev hloov pauv caj ces (JAK2, CALR, MPL) los tsis muaj.

Qhov tseem ceeb tshaj plaws yog tham nrog koj tus kws kho mob txog seb koj qhov kev kuaj mob yuav cuam tshuam li cas rau koj lub neej.

Kuv yuav saib xyuas kuv tus kheej li cas? (Kev saib xyuas tus kheej)

Nug koj tus kws kho mob seb koj puas yuav tau txais txiaj ntsig los ntawm kev kho mob palliative . Cov pab pawg kho mob palliative yuav suav nrog cov kws kho mob, cov kws tu mob, cov neeg ua haujlwm pabcuam zej zog, thiab lwm tus kws tshwj xeeb. Lawv tuaj yeem muab cov peev txheej thiab kev txhawb nqa rau koj uas koj xav tau los nyob nrog tus kab mob no. Ntxiv nrog rau kev kho mob uas koj tau txais los ntawm koj tus kws kho mob qog noj ntshav, cov neeg no tuaj yeem yog qhov chaw zoo ntawm kev txhawb nqa. Cov kws tshwj xeeb kho mob palliative tuaj yeem pab txhim kho koj lub neej zoo li tus neeg uas muaj mob qog noj ntshav.

Thaum kawg, tej yam tseem ceeb tshaj plaws rau koj yuav tsum nco ntsoov

Myelofibrosis yog ib hom mob qog ntshav tsawg tsawg uas koj cov pob txha hloov los ntawm cov ntaub so ntswg fibrous. Qhov no yog ib qho mob hnyav uas yuav tsum tau saib xyuas thiab/lossis kho mob tas li. Nyob ntawm koj tus mob, koj yuav tsis muaj tsos mob li ntau xyoo. Hauv lwm qhov xwm txheej, cov tsos mob tuaj yeem nce qib sai thiab ua rau nws nyuaj rau ua cov haujlwm txhua hnub. Txawm li cas los xij, muaj kev kho mob los pab tswj cov tsos mob uas cuam tshuam rau koj lub neej txhua hnub.

Lub sijhawm no, nug koj tus kws kho mob txog cov peev txheej uas tuaj yeem pab koj daws cov kev hloov pauv no. Kev saib xyuas palliative thiab cov pab pawg txhawb nqa yog cov kev xaiv pab tau zoo heev thaum koj hloov kho rau lub neej nrog mob qog noj ntshav.

Nco ntsoov tias, koj tsis nyob ib leeg xwb. Cov kws kho mob, tsev neeg, thiab cov phooj ywg puav leej nyob ntawd los pab koj. Tsis txhob ntshai tham nrog koj tus kws kho mob txog tej lus nug uas koj muaj.

Myelofibrosis , mob qog ntshav, pob txha pob txha, ntshav tsis txaus, lub qog loj hlob, JAK inhibitors, mob ntshav dawb ntau ntau