Ingaba umongo wakho wethambo uvelisa izicubu ezine-fibrous? Masifunde nge-myelofibrosis!

Ngaba wakha weva ngesifo esibizwa ngokuba yi-myelofibrosis? Ngokwenene luhlobo olungaqhelekanga lomhlaza wegazi. Ngamafutshane, kuxa umongo wamathambo , oyinxalenye ethambileyo nethambileyo yamathambo akho, uthatyathelw’ indawo yizicubu ezibomvu . Kwakhona luhlobo lwe-leukemia yexesha elide, kwaye lukwiqela elibizwa ngokuba yi-myeloproliferative disorders. Iingxaki ze-Myeloproliferative zixa iiseli zegazi ezininzi kakhulu ziveliswa kumongo wamathambo, apho kwenziwa khona iiseli zegazi.

Yintoni kanye kanye i-myelofibrosis?

Masijonge oku ngokweenkcukacha ezithe vetshe. Umongo wakho wethambo uneeseli ze- stem ezakha igazi. Ezi seli ze-stem kamva zikhula zibe ziiseli ezibomvu zegazi, iiseli ezimhlophe zegazi, kunye neeplatelets. Kodwa kumntu one-myelofibrosis, utshintsho lwenzeka kwizinto zemfuza, okanye i-DNA, kwenye yezi seli ze-stem. Oku kubangela ukuba iseli ibe yiseli yomhlaza enesiphako. Le seli inesiphako emva koko yahlukana ize idlulisele utshintsho kuzo zonke iiseli ezintsha ezenzayo.

Ekuhambeni kwexesha, ezi seli zomhlaza ezingaqhelekanga ziyaqokelelana ngamanani amakhulu. Ezinye zezi seli zibangela ukudumba kumongo wethambo. Oku kudumba kuko okubangela ukuba kubekho izicubu ezibomvu endizikhankanyileyo ngaphambili. Ngoko ke, ngenxa yesi sikhumba kunye neeseli zomhlaza ezigqithisileyo, umongo wethambo ulahlekelwa ngamandla awo okwenza iiseli zegazi eziphilileyo.

Ziziphi iintlobo eziphambili ze-myelofibrosis?

Kukho iintlobo ezimbini eziphambili ze-myelofibrosis.

• I-myelofibrosis ephambili: Olu luhlobo oluvela ngokungazi, ngaphandle kokunxibelelana naso nasiphi na esinye isifo.
• I-myelofibrosis yesibini: Oku kubangelwa yenye ingxaki yegazi. Umzekelo, inokubangelwa zizifo ezifana ne-primary thrombocytosis okanye i-polycythemia vera. Olu hlobo lwesibini lubandakanya phakathi kwe-10% kunye ne-20% yezigulana ezifunyaniswe ukuba zineengxaki.

Ixhaphake kangakanani le sifo ibizwa ngokuba yiMyelofibrosis?

I-Myelofibrosis yimeko engaqhelekanga kakhulu . Umzekelo, eMelika, kubikwa ukuba esi sifo senzeka kubantu abamalunga ne-1.5 kwabayi-100,000 ngonyaka. Sinokuchaphazela nabani na, nokuba uneminyaka emingaphi. Akukho mahluko ngeminyaka, kodwa kunokwenzeka ukuba sifumaneke kubantu abangaphezulu kweminyaka engama-50. Ukuba i-myelofibrosis ikhula kubantwana abancinci, idla ngokufunyaniswa ingekafiki iminyaka emi-3.

Ziziphi iziphumo ezinokubakho emzimbeni wakho ngenxa ye-myelofibrosis?

Xa iiseli zegazi ziveliswa ngendlela engaqhelekanga ngale ndlela, iingxaki ezahlukeneyo zinokwenzeka. Makhe sijonge ukuba zeziphi:

• I-anemia: Le yimeko apho iiseli ezibomvu zegazi zinganeli. Njengoko usazi, iiseli ezibomvu zegazi zezona zithwala ioksijini emzimbeni wonke. Ngoko ke xa iiseli ezibomvu zinganeli, izicubu zomzimba azifumani ioksijini eyaneleyo. Oku kunokubangela iimpawu ezifana nokudinwa, ubuthathaka, kunye nokuphefumla nzima .

• I-Thrombocytopenia: Le yimeko apho iiplatelets zimbalwa kakhulu. Iiplatelets zezona zinceda ukujiya kwegazi xa kukho ukwenzakala. Ngoko ke xa inani leeplatelets zakho liphantsi, umzimba wakho unokuba semngciphekweni wokopha nokukrweleka .

• I-Splenomegaly: I-spleen yakho ilawula ukubalwa kweeseli zegazi kwaye isusa iiseli ezibomvu zegazi ezonakeleyo emzimbeni wakho. Ngoko ke xa usenza iiseli zegazi ezininzi ezingaqhelekanga, i-spleen yakho kufuneka isebenze nzima. Ingakhula. Xa i-spleen yakho ikhula, ungaziva ugcwele okanye ungakhululeki kwinxalenye ephezulu yasekhohlo yesisu sakho .

• I-Extramedullary hematopoiesis: Oku kukukhula kweeseli ezenza igazi ngaphandle komongo wethambo , kwezinye iindawo zomzimba. Ezi seli zinokuvela kwiindawo ezifana nemiphunga, indlela yokugaya ukutya, umqolo, ubuchopho, okanye ii-lymph nodes. Ezi seli zinokuhlangana ukuze zenze iithumba , ezinokungena kwamanye amalungu omzimba okanye ziphazamise ukusebenza kwazo.

• Uxinzelelo lwegazi oluphezulu kwi-portal: Oku kukunyuka koxinzelelo lwegazi kwimithambo yegazi ethwala igazi lisuka kwi-spleen yakho ukuya esibindini sakho. Oku kusenokwenzeka ukuba kubangelwa kukonakala kwemithambo yegazi ngenxa ye-extramedullary hematopoiesis ekhankanyiweyo ngaphambili.

Enye into kukuba malunga ne-12% yabantu abane-primary myelofibrosis, le meko inokuba luhlobo olubi kakhulu lomhlaza wegazi olubizwa ngokuba yi -acute myeloid leukemia .

Yintoni ebangela i-myelofibrosis?

Izazinzulu azikayazi kakuhle into ebangela i-myelofibrosis. Kodwa zifumanise ukuba inxulumene notshintsho kwi-DNA yezakhi ezithile zemfuza . Uhlobo lweproteni olubizwa ngokuba yi -Janus-associated kinases (JAKs) lubandakanyeka. Ezi proteni ze-JAK zilawula ukuveliswa kweeseli zegazi kumongo wethambo, zithumela imiqondiso kwiiseli ukuba zahlukane kwaye zikhule. Ukuba ezi proteni ze-JAK zisebenza kakhulu, iiseli zegazi ezininzi okanye ezimbalwa kakhulu zinokuveliswa.

Phakathi kwama-60% nama-65% abantu abane-myelofibrosis banotshintsho kwi-JAK2 gene . Enye i-5% ukuya kwi-10%Kukho utshintsho kwi-myeloproliferative leukemia (MPL) gene. Kwakhona, utshintsho olubizwa ngokuba yiCalreticulin (CALR) lubonwa phakathi kwe-20% kunye ne-25% yezigulane ze-myelofibrosis.

Ngubani osengozini enkulu yoku?

Usenokuba sengozini enkulu yokuphuhlisa i-myelofibrosis ngenxa yezi zizathu zilandelayo:

• Ukuba uneminyaka engaphezu kwama-50 ubudala .
• Ukuba unesifo segazi esibizwa ngokuba yi-primary thrombocytosis okanye i-polycythemia vera.
• Ukuba ukhe wachatshazelwa yimitha ye-ionizing okanye iikhemikhali zepetrochemicals ezifana ne-benzene kunye ne-toluene.

Ziziphi iimpawu ze-myelofibrosis?

I-Myelofibrosis sisifo esiqhubeka kancinci kancinci , ngoko ke usenokungabi nazimpawu kangangeminyaka. Malunga nesithathu kwisithathu sezigulane azinazo iimpawu kwisigaba sokuqala.

Ukuba iimpawu ziyenzeka, ezona zixhaphakileyo kukudinwa kakhulu (ngenxa ye-anemia) kunye nokwanda kwepleen . Iimpawu zingabandakanya:

• Umsebenzi onzima
• Ifiva
• Ukurhawuzelela
• Ulusu olukhanyayo
• Ukuhla ukusinda
• Ukubila ebusuku
• Intlungu yamathambo okanye yamalungu
• Usulelo oluqhelekileyo
• Isibindi okanye ipleyini ekhulisiweyo
• Amahlwili egazi angachazwanga
• Ukopha okungaqhelekanga okanye ukukrweleka
• Ukwanda kwemithambo esiswini nasemqaleni (le mithambo inokuqhuma ize yophe igazi).

Indlela yokuxilonga esi sifo?

Ugqirha, ingakumbi ingcali yomhlaza, uza kukuhlola ngokwasemzimbeni, akubuze ngembali yakho yezonyango, aze akubuze ngeempawu zakho zangoku. Baza kujonga nokuba ipleen ikhulisile na kunye neempawu ze-anemia.

Emva koko, kwenziwa iimvavanyo ezahlukeneyo ukuze kuqinisekiswe ukuba akukho zimo zimbi kwaye kuqinisekiswe ukuba unayo na i-myelofibrosis.

Uvavanyo lwegazi

• Ukubalwa kwegazi okupheleleyo (CBC): Oku kulinganisa inani leentlobo ezahlukeneyo zeeseli egazini lakho. Ukuba iiseli zakho ezibomvu zegazi ziphantsi kunesiqhelo, okanye iiseli zakho ezimhlophe zegazi kunye neeplatelets aziqhelekanga, oko kungaba luphawu lwe-myelofibrosis.
• I-Peripheral blood smear (PBS): Oku kujonga ubungakanani, imilo, kunye nezinye iimpawu zeeseli zakho zegazi ukuba azinazo iimpawu ezingaqhelekanga na. Ukuba kukho iiseli ezibonakala zingaqhelekanga kunye nenani elikhulu leeseli zegazi ezingavuthwanga, isenokuba luphawu lwe-myelofibrosis.
• Uvavanyo lwekhemikhali yegazi:Olu vavanyo lulinganisa amanqanaba ezinto ezahlukeneyo ezikhutshwa egazini ezivela kwizitho zakho zomzimba. Oku kunokukuxelela ukuba isitho sisebenza kakuhle kangakanani. Amanqanaba aphezulu ezinto ezifana ne-uric acid, i-bilirubin, kunye ne-lactic dehydrogenase egazini nawo angaba luphawu lwe-myelofibrosis.

Uvavanyo lomongo wethambo

• I-Bone Marrow Biopsy: Oku kuquka ukuthatha isampuli encinci yomongo wakho wethambo uze uwuhlole phantsi kwe-microscope. Iiseli ziyahlolwa ukuze kubonwe ukuba unayo na i-myelofibrosis.
• Ukufunxwa komongo wethambo: Oku kuquka ukususa ulwelo kumongo wethambo nokuluvavanya ukuze kubonwe iimpawu ze-myelofibrosis.

Kusenokufuneka iimvavanyo ezingaphezulu

Kunokufuneka iimvavanyo ezongezelelweyo ukuqinisekisa ukuxilongwa kwakho:

• Uhlalutyo lokuguquka kwezakhi zofuzo: Ugqirha wakho uza kuvavanya iiseli zakho zegazi kunye nethambo lomongo ukuze ajonge utshintsho lwezakhi zofuzo oluhambelana ne-myelofibrosis, njenge-JAK2, i-CALR, kunye ne-MPL. Ezinye iindlela zonyango zijolise kwiiseli zomhlaza ezine-JAK2 mutation.
• Iinkqubo zokuthatha imifanekiso: Ugqirha wakho unokwenza i-ultrasound ukujonga ukuba i-spleen inkulu na. Banokwenza ne-MRI ukujonga ukuba i-scle tissue kwi-bone mongo. Le scle tissue inokuba luphawu lwe-myelofibrosis.

Inyangwa njani i-myelofibrosis?

Ukuba awubonisi zimpawu, usenokungadingi unyango. Nangona kunjalo, nokuba awudingi unyango olukhawulezileyo, ugqirha wakho uya kuqhubeka ekujonga imeko yakho.

I-Jakafi® (ruxolitinib), i-Inrebic® (fedratinib), kunye ne-Vonjo® (pacritinib) ngamayeza avunyiweyo yi-U.S. Food and Drug Administration (FDA) . Asetyenziselwa ukunyanga i-myelofibrosis enomngcipheko ophakathi ukuya kophezulu. Omathathu la mayeza asebenza njenge -JAK inhibitors . Oko kukuthi, anciphisa i-Janus-associated kinase (JAK) signaling esebenzayo kakhulu. Ngenxa yoko, la mayeza anceda ekunciphiseni iimpawu ezinxulumene ne-myelofibrosis, ezinje nge-spleen ekhulisiweyo, ukubila ebusuku, ukurhawuzelela, ukwehla kobunzima, kunye nomkhuhlane.

Kwabaninzi, injongo ephambili yonyango kukulawula iimeko ezinxulumene ne-myelofibrosis, njenge-anemia kunye ne-spleen ekhulisiweyo.

Unyango lwe-Anemia

Kukho iindlela zonyango lwe-anemia ezifana nezi:

• I-Androgens: Ukuthatha ii-androgens ezifana ne-danazol kunokunyusa imveliso yeeseli ezibomvu zegazi.
• Ii-immunomodulators: La mayeza andisa amandla omzimba wakho okulwa neeseli zomhlaza, ngaloo ndlela enciphisa iimpawu. Amayeza afana ne-interferon, i-thalidomide (Thalomid®), kunye ne-lenalidomide (Revlimid®) akweli qela. Anganikwa kunye ne-glucocorticoids efana ne-prednisone.
• Amayeza e-chemotherapy: Amanye amayeza e-chemotherapy anokunciphisa iimpawu ezibangelwa kukwanda kweeseli zegazi kunye nokwanda kwe-spleen. I-Hydroxyurea kunye ne-cladribine zezo ziyobisi.
• Utofelo-gazi: Ukuba une-anemia enzima, utofelo-gazi oluqhelekileyo lunokunyusa inani leeseli zakho ezibomvu zegazi.

Unyango lweSplenomegaly

Ii-JAK inhibitors, ii-immunomodulators, kunye namayeza e-chemotherapy asetyenziselwa ukulawula i-spleen ekhulisiweyo. Kwiimeko ezinzima, kunokufuneka ukuba i-spleen yakho isuswe ngotyando (i-spleenectomy) okanye ufumane unyango lwe-radiation kwi-spleen.

Unyango ngemitha lunokufuneka ukunyanga imeko ebizwa ngokuba yi-extramedullary hematopoiesis, apho iiseli zegazi zikhula ngaphandle komongo wethambo.

Ngaba i-myelofibrosis inganyangeka ngokupheleleyo?

Ukufakelwa kweeseli ze-hematopoietic ze-allogeneic (HCT) kusenokuba lolona khetho lokunyanga esi sifo. Nangona kunjalo, le yinkqubo enobungozi kwaye ayifanelekanga kuye wonke umntu.

Kule nkqubo, iiseli zakho zegazi ezingaqhelekanga zitshintshwa ziiseli ezivela kumntu ophilileyo. Ngaphambi kokufakelwa, uza kunikwa i-chemotherapy okanye unyango lwe-radiation ukubulala iiseli zakho ezigulayo. Emva koko, iiseli ezintsha ezivela kumntu onikelayo zingathatha imisebenzi yomzimba wakho.

Unyango lwe-HCT lunomngcipheko omkhulu weengxaki . Ke ngoko, lufanelekela kuphela iqela elithile labantu. Umngcipheko weengxaki uphezulu nangakumbi kubantu abanezinye izifo. Ukuba ufanelekile oku kuxhomekeke kwizinto ezininzi, ezinje ngobudala bakho, ubukhali beempawu zakho, kunye nethuba lokuphumelela konyango.

Injani ixesha lokuphila xa uphila nesi sifo?

I-Myelofibrosis ngumhlaza onzima kakhulu . Izinga lokusinda eliphakathi kwaba baguli lidla ngokuba malunga neminyaka emithandathu. Izinga lokusinda eliphakathi lithetha ukuba abanye abantu baphila ngaphantsi kweminyaka emithandathu, ngelixa inani elifanayo liphila ngaphezu kweminyaka emithandathu. Oku akufani kuwo wonke umntu, kuyahluka kumntu nomntu.

Kukho izinto ezininzi ezichaza ukuba isifo sakho siza kuqala nini:

• iminyaka yakho.
• Iimpawu zakho.
• Inani leeseli zakho zegazi.
• Ubukhali bokuqhekeka kwamanxeba kumongo wethambo.
• Nokuba kukho utshintsho lwezakhi zofuzo okanye akunjalo (JAK2, CALR, MPL).

Eyona nto ibalulekileyo kukuthetha ngokukhululekileyo nogqirha wakho malunga nendlela ukuxilongwa kwakho okuya kuyichaphazela ngayo ubomi bakho.

Ndingazinyamekela njani? (Ukuzinyamekela)

Buza ugqirha wakho ukuba ungazuza na kunyango lokudambisa iintlungu . Amaqela onyango lokudambisa iintlungu angabandakanya oogqirha, abahlengikazi, abasebenzi bezentlalo, kunye nezinye iingcali. Banokukunika izixhobo kunye nenkxaso oyifunayo ukuze uphile nesi sifo. Ukongeza kunyango olufumana kwingcali yakho yomhlaza, aba bantu banokuba ngumthombo omkhulu wenkxaso. Iingcali zonyango lokudambisa iintlungu zinokukunceda ukuphucula umgangatho wobomi bakho njengomntu ophila nomhlaza.

Okokugqibela, ezona zinto zibalulekileyo ekufuneka uzikhumbule

I-Myelofibrosis luhlobo olungaqhelekanga lomhlaza wegazi apho umongo wakho wethambo uthatyathelwa indawo yizicubu ezine-fibrous. Le yimeko enzima efuna ukubekwa esweni rhoqo ngugqirha kunye/okanye unyango. Ngokuxhomekeke kwimeko yakho, usenokungabi nazimpawu kangangeminyaka. Kwezinye iimeko, iimpawu zinokukhula ngokukhawuleza kwaye zenze kube nzima ukwenza imisebenzi yemihla ngemihla. Nangona kunjalo, kukho unyango olufumanekayo ukunceda ukulawula iimpawu eziphazamisana nobomi bakho bemihla ngemihla.

Okwangoku, buza ugqirha wakho ngezixhobo ezinokukunceda ujongane nolu tshintsho. Unyango oluthomalalisayo kunye namaqela enkxaso zizinto eziluncedo kakhulu njengoko uqhelana nobomi obunomhlaza.

Khumbula, awuwedwa. Oogqirha, usapho, kunye nabahlobo bakho bonke ukukunceda. Musa ukoyika ukuthetha nogqirha wakho ngayo nayiphi na imibuzo onokuba nayo.

I- Myelofibrosis, umhlaza wegazi, umongo wamathambo, i-anemia, ukwanda kwe-spleen, izithinteli ze-JAK, i-leukemia